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Anemia Hemolítica Autoinmune

La anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune es un tipo raro de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica que se caracteriza por la producción de anticuerpos contra los LOS Neisseria eritrocitos propios, lo que provoca la destrucción de estas células en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el bazo y otros tejidos reticuloendoteliales. La enfermedad se clasifica generalmente como caliente o fría, dependiendo de la reactividad térmica de los LOS Neisseria autoanticuerpos. La etiología es diversa e incluye infecciones, enfermedades autoinmunes, trastornos linfoproliferativos y medicamentos. El diagnóstico se basa en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum pruebas de laboratorio de hemólisis ( LDH LDH Osteosarcoma y bilirrubina indirecta elevada, haptoglobina reducida) en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum asociación con una prueba de antiglobulina directa positiva o test de Coombs. El tratamiento implica terapia de soporte, la eliminación del agente causante, uso de esteroides y agentes inmunosupresores, y esplenectomía en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria casos refractarios.

Last updated: Dec 15, 2025

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Descripción General

Definición

La anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune es la hemólisis o destrucción prematura de los LOS Neisseria eritrocitos por autoanticuerpos. Existen dos categorías de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types autoinmune basadas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la reactividad térmica de los LOS Neisseria autoanticuerpos:

  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes
  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos

Epidemiología

  • 1–3 casos por cada 100 000 habitantes al AL Amyloidosis año
  • Es ligeramente más probable que se produzca en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las mujeres que en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria hombres
  • Causa secundaria presente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum 20%–80% de los LOS Neisseria casos reportados
  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes:
    • 70%–80% de las anemias hemolíticas autoinmunes
    • Se observa en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum niños de 0–4 años y en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum adultos de > 40 años
  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos: incidencia máxima en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum adultos > 60 años

Etiología

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes:

  • Idiopática/primaria: sin enfermedad subyacente ni medicamentos
  • Secundaria: asociada a una enfermedad subyacente (50%–60% de los LOS Neisseria casos)
    • Trastornos linfoproliferativos
    • Trastornos autoinmunes
    • Malignidad
    • Inmunodeficiencia
    • Infecciones
    • Embarazo
    • Medicamentos
    • Trasplante alogénico de células madre hematopoyéticas: normalmente entre 2–6 meses después del injerto

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos:

  • Idiopática/primaria (enfermedad por crioaglutininas)
  • Síndrome por crioaglutininas:
    • Trastornos linfoproliferativos
    • Infecciones
    • Enfermedad autoinmune
    • Malignidad
  • Hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions paroxística por frío (observada casi exclusivamente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum niños):
    • Idiopática
    • Secundaria (puede ser aguda transitoria o crónica)

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune de tipo mixto:

  • Idiopática
  • Secundaria (trastornos linfoproliferativos, trastornos autoinmunes)

Causas específicas de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune

Tabla: Causas específicas de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes (principalmente anticuerpos IgG IgG The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of igg, for example, igg1, igg2a, and igg2b. Hypersensitivity Pneumonitis)
Tipo Causa específica
Primaria Idiopática
Secundaria Infección viral VIH
Otras enfermedades/condiciones
  • Malignidad
  • Trastornos linfoproliferativos (e.g., leucemia linfocítica crónica)
  • Trastornos autoinmunes (e.g., lupus eritematoso sistémico)
  • Trasplante alogénico de células madre hematopoyéticas
  • Embarazo
Medicamentos
  • Penicilina
  • Ceftriaxona
  • Metildopa
Tabla: Causas específicas de la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos (principalmente anticuerpos IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions)
Tipo Causa específica
Primaria Enfermedad por crioaglutininas primaria
Secundaria Infección viral
  • EBV EBV Epstein-barr virus (EBV) is a linear, double-stranded DNA virus belonging to the herpesviridae family. This highly prevalent virus is mostly transmitted through contact with oropharyngeal secretions from an infected individual. The virus can infect epithelial cells and B lymphocytes, where it can undergo lytic replication or latency. Epstein-Barr Virus
  • CMV
  • La hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions paroxística por frío se presenta comúnmente después de una infección viral en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria niños.
Infección bacteriana
  • Mycoplasma pneumoniae Mycoplasma pneumoniae Short filamentous organism of the genus mycoplasma, which binds firmly to the cells of the respiratory epithelium. It is one of the etiologic agents of non-viral primary atypical pneumonia in man. Mycoplasma
  • La hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions paroxística por frío también se asocia con Klebsiella Klebsiella Klebsiella are encapsulated gram-negative, lactose-fermenting bacilli. They form pink colonies on MacConkey agar due to lactose fermentation. The main virulence factor is a polysaccharide capsule. Klebsiella pneumoniae is the most important pathogenic species. Klebsiella, Escherichia coli Escherichia coli The gram-negative bacterium Escherichia coli is a key component of the human gut microbiota. Most strains of E. coli are avirulent, but occasionally they escape the GI tract, infecting the urinary tract and other sites. Less common strains of E. coli are able to cause disease within the GI tract, most commonly presenting as abdominal pain and diarrhea. Escherichia coli y sífilis (congénita y terciaria)
Otras condiciones
  • Trastornos linfoproliferativos
  • Malignidad
  • Trastornos autoinmunes
Medicamentos Lenalidomida (rara)

Fisiopatología

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes

  • Los LOS Neisseria autoanticuerpos calientes son responsables de la mayoría de los LOS Neisseria casos de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune
  • Los LOS Neisseria anticuerpos (predominantemente IgG IgG The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of igg, for example, igg1, igg2a, and igg2b. Hypersensitivity Pneumonitis) interactúan con los LOS Neisseria antígenos del complejo del factor Rhesus o los LOS Neisseria antígenos glicoproteicos.
  • Los LOS Neisseria autoanticuerpos calientes reaccionan más fuertemente a 37°C.
  • Patogénesis:
    • En EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la mayoría de los LOS Neisseria casos, la hemólisis (facilitada por las células fagocíticas) se produce de forma extravascular.
    • La destrucción de los LOS Neisseria eritrocitos se produce donde los LOS Neisseria macrófagos son abundantes, sobre todo en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el bazo (también en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el hígado y la médula ósea):
      • La porción Fc Fc Crystallizable fragments composed of the carboxy-terminal halves of both immunoglobulin heavy chains linked to each other by disulfide bonds. Fc fragments contain the carboxy-terminal parts of the heavy chain constant regions that are responsible for the effector functions of an immunoglobulin (complement fixation, binding to the cell membrane via fc receptors, and placental transport). This fragment can be obtained by digestion of immunoglobulins with the proteolytic enzyme papain. Immunoglobulins: Types and Functions (anticuerpo) es reconocida por el receptor Receptor Receptors are proteins located either on the surface of or within a cell that can bind to signaling molecules known as ligands (e.g., hormones) and cause some type of response within the cell. Receptors Fc Fc Crystallizable fragments composed of the carboxy-terminal halves of both immunoglobulin heavy chains linked to each other by disulfide bonds. Fc fragments contain the carboxy-terminal parts of the heavy chain constant regions that are responsible for the effector functions of an immunoglobulin (complement fixation, binding to the cell membrane via fc receptors, and placental transport). This fragment can be obtained by digestion of immunoglobulins with the proteolytic enzyme papain. Immunoglobulins: Types and Functions de los LOS Neisseria fagocitos → fagocitosis de la cadena pesada de la IgG IgG The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of igg, for example, igg1, igg2a, and igg2b. Hypersensitivity Pneumonitis y parte de la membrana celular → se forma el esferocito
      • Células T citotóxicas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el bazo → citotoxicidad mediada por células → hemólisis de eritrocitos
      • Los LOS Neisseria macrófagos del hígado reconocen los LOS Neisseria fragmentos del complemento C3 → Los LOS Neisseria eritrocitos con complemento son fagocitados.
  • Más del 95% de los LOS Neisseria casos de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes tienen una prueba de antiglobulina directa o test de Coombs positiva.
Anemia hemolítica inducida por medicamentos

Anemia hemolítica autoinmune inducida por medicamentos:
Los medicamentos pueden causar anemia hemolítica autoinmune por:
Arriba: unión de un medicamento a proteínas específicas que permanecen en la superficie de los eritrocitos y son reconocidos como nuevos antígenos por las IgG (penicilina)
Medio: Activación del complemento inducida por complejos inmunes formados por un medicamento(quinidina)
Abajo: Alteración de la molécula del factor Rhesus (Rh) o de un componente de la membrana celular por un medicamento, lo que lo hace inmunogénico para la IgG incluso después de la interrupción del medicamento (metildopa).

Imagen por Lecturio.

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos

Los LOS Neisseria autoanticuerpos sensibles al AL Amyloidosis frío reaccionan más fuertemente a 0–4℃.

  • Aglutininas frías:
    • Los LOS Neisseria anticuerpos (predominantemente IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions) interactúan con el antígeno de eritrocitos I o con el antígeno i por debajo de la temperatura central normal:
      • Antígeno I: presente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el 99% de la población
      • Antígeno i: observado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum < 1%
    • Tras unirse a los LOS Neisseria eritrocitos, los LOS Neisseria autoanticuerpos IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions activan la cascada clásica del complemento (C1 → C3b).
    • Los LOS Neisseria eritrocitos recubiertos por C3b son sometidos a fagocitosis extravascular ( en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el sistema reticuloendotelial, principalmente por las células de Kupffer en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el hígado)
    • Resulta en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum hemólisis extravascular
    • Al AL Amyloidosis calentarse, la IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions se disocia, pero la C3b permanece y, por tanto, se detecta en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la prueba de antiglobulina directa.
  • Anticuerpos Donath-Landsteiner:
    • Reconocen los LOS Neisseria antígenos de los LOS Neisseria eritrocitos (a menudo el antígeno P) en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum frío y fijan el complemento, dando lugar a la hemólisis
    • Hemólisis intravascular → hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions y anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
    • Encontrado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions paroxística por frío

Presentación Clínica

  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types:
    • Disnea
    • Taquicardia
    • Fatiga
    • Palidez
    • Palpitaciones
  • Hemólisis extravascular:
    • Ictericia
    • Cálculos biliares
    • Esplenomegalia
    • Hepatomegalia
    • Linfadenopatía
  • Hemólisis intravascular:
    • Orina oscura ( hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions)
    • Insuficiencia renal transitoria

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos:

  • Acrocianosis:
    • Coloración gris oscura o púrpura de los LOS Neisseria dedos de los LOS Neisseria pies, las orejas o la nariz al AL Amyloidosis exponerse al AL Amyloidosis frío
    • Puede provocar gangrena en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum casos graves
  • Fenómeno de Raynaud
  • Livedo reticularis Livedo reticularis A condition characterized by a reticular or fishnet pattern on the skin of lower extremities and other parts of the body. This red and blue pattern is due to deoxygenated blood in unstable dermal blood vessels. The condition is intensified by cold exposure and relieved by rewarming. Chronic Kidney Disease: piel pálida con un patrón reticulado azulado o violáceo
Cambio gangrenoso y acrocinosis

Acrocianosis de las manos:
Acrocianosis con gangrena (A) y resolución de la acrocianosis (B)

Imagen: “Gangrenous change and acrocynosis” por Department of Internal Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan. Licencia: CC BY 4.0

Diagnóstico

Evaluación inicial de laboratorios

El diagnóstico se realiza por evidencia de hemólisis y test de Coombs directo positivo.

  • Hemograma:
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
    • El volumen corpuscular medio (VCM) varía con el recuento de reticulocitos
    • Recuento de leucocitos y plaquetas generalmente normal
  • Recuento de reticulocitos: suele estar aumentado
  • Frotis de sangre periférica:
    • Policromasia (debido a un recuento elevado de reticulocitos)
    • Esferocitos
    • Esquistocitos
    • Eritrocitos nucleados
    • Grupos de eritrocitos
  • Test de Coombs directo:
    • Establece el diagnóstico
    • Identifica la presencia de anticuerpos y/o complemento unidos a los LOS Neisseria eritrocitos
    • La fuerza de la prueba positiva se correlaciona con la gravedad de la hemólisis.
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes:
      • Positivo a 37°C para anti-IgG
      • El anti-C3 puede ser positivo o no.
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos: positivo para anti-C3 y negativo para anti-IgG
  • Marcadores séricos de hemólisis:
    • ↑ Bilirrubina indirecta
    • LDH LDH Osteosarcoma
    • AST AST Enzymes of the transferase class that catalyze the conversion of l-aspartate and 2-ketoglutarate to oxaloacetate and l-glutamate. Liver Function Tests y ALT ALT An enzyme that catalyzes the conversion of l-alanine and 2-oxoglutarate to pyruvate and l-glutamate. Liver Function Tests
    • Haptoglobina sérica baja
  • Los LOS Neisseria niveles de C3 y C4 se reducen en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos
  • Título de crioaglutininas: título elevado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos

Evaluación adicional

  • Análisis de orina, con tira reactiva y microscopio:
    • Hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions → tira reactiva positiva para sangre pero ausencia de eritrocitos
    • Análisis de orina normal en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la hemólisis extravascular
  • Nitrógeno de urea Urea A compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. Urea Cycle/creatinina: deterioro renal en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la hemólisis intravascular
  • Revisar las posibles causas secundarias:
    • Condiciones subyacentes
    • Infecciones recientes (i.e, pruebas serológicas positivas para Mycoplasma pneumoniae Mycoplasma pneumoniae Short filamentous organism of the genus mycoplasma, which binds firmly to the cells of the respiratory epithelium. It is one of the etiologic agents of non-viral primary atypical pneumonia in man. Mycoplasma)
    • Medicamentos
    • Transfusión
  • Aspiración de médula ósea en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum indicaciones relevantes (i.e., para excluir leucemia linfocítica crónica)

Tratamiento

Principios del tratamiento

  • Estabilización del paciente:
    • Evaluar la gravedad de la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types y los LOS Neisseria síntomas asociados (i.e., disnea o síntomas asociados a isquemia cardíaca).
    • Apoyo hemodinámico y ventilatorio según sea necesario
    • Determinar la necesidad de transfusión (transfundir cuando sea necesario).
    • Suspender el medicamento sospechoso.
  • Se recomienda el tratamiento de la enfermedad subyacente, si corresponde.
  • La hemólisis continuará hasta 3 semanas (equivalente a la vida media de los LOS Neisseria autoanticuerpos).

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes

  • Suplemento de ácido fólico: La deficiencia de folato puede desarrollarse con la hemólisis crónica.
  • Glucocorticoides +/- rituximab Rituximab A murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis. Immunosuppressants:
    • Puede comenzarse con ambos como tratamiento inicial, O
    • Iniciar rituximab Rituximab A murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis. Immunosuppressants si no hay respuesta con glucocorticoides después de 2–3 semanas
  • Inmunoglobulinas intravenosas
  • Enfermedad refractaria:
    • Inmunosupresores: micofenolato, ciclofosfamida o azatioprina
    • Esplenectomia

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos

  • Evitar el frío.
  • Suplementación con ácido fólico
  • Inmunosupresores: regímenes que contienen rituximab Rituximab A murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis. Immunosuppressants (i.e., rituximab Rituximab A murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis. Immunosuppressants con bendamustina)
  • Plasmaféresis en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum casos de hemólisis grave

Complicaciones y pronóstico

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos calientes:

  • ↑ Riesgo de tromboembolismo venoso:
    • Descartar trombosis venosa profunda si el dímero-D es positivo.
    • Descartar una embolia pulmonar si la disnea es desproporcionada respecto a la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types.
  • Síndrome de Evans:
    • Se producen 2 o más citopenias inmunitarias simultáneamente.
    • La anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune puede presentarse con púrpura trombocitopénica inmune (PTI).
    • Asociado a una mayor tasa de mortalidad
  • La mayoría de los LOS Neisseria casos responden a la terapia en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum 2–3 semanas.
  • La recaída es posible, y los LOS Neisseria pacientes deben ser instruidos para buscar atención médica si los LOS Neisseria síntomas se repiten.

Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune por anticuerpos fríos:

  • Supervivencia similar a la de las poblaciones emparejadas por edad
  • En EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la hemoglobinuria Hemoglobinuria The presence of free hemoglobin in the urine, indicating hemolysis of erythrocytes within the vascular system. After saturating the hemoglobin-binding proteins (haptoglobins), free hemoglobin begins to appear in the urine. Transfusion Reactions paroxística por frío asociada a virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology que afecta a los LOS Neisseria niños, la recuperación se observa en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum 2 semanas.
  • Los LOS Neisseria episodios recurrentes son más frecuentes en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria adultos.

Diagnóstico Diferencial

  • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica: grupo de anemias que se deben a la destrucción o eliminación prematura de los LOS Neisseria eritrocitos. La hemólisis puede ser intravascular o extravascular ( en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el bazo). La etiología de la destrucción prematura de eritrocitos es diversa y puede deberse a condiciones como defectos de la membrana de los LOS Neisseria eritrocitos, defectos enzimáticos de los LOS Neisseria eritrocitos, destrucción inmunitaria de los LOS Neisseria eritrocitos, lesiones mecánicas e hiperesplenismo. En EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las causas no autoinmunes de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica, el test de Coombs directo es característicamente negativo.
  • Lupus eritematoso sistémico (LES): enfermedad crónica, autoinmune e inflamatoria que provoca el depósito de inmunocomplejos en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria órganos, lo que da lugar a manifestaciones sistémicas. Esta enfermedad afecta mayoritariamente a las mujeres, sobre todo a las de origen afroamericano. Las características clínicas más notables incluyen una erupción malar, artritis no destructiva, nefritis lúpica, serositis Serositis Inflammation of a serous membrane. Systemic Lupus Erythematosus, citopenias, enfermedad tromboembólica, convulsiones y/o psicosis. El diagnóstico se realiza mediante el cumplimiento de los LOS Neisseria criterios clínicos, que incluyen anticuerpos antinucleares, anticuerpos específicos del LES y hallazgos clínicos específicos.
  • Leucemia linfocítica crónica: la leucemia más común en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum adultos. La leucemia linfocítica crónica es una proliferación monoclonal de células B que se infiltra en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la médula ósea o en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria ganglios linfáticos. Las células B se detienen antes de la maduración, por lo que estos pacientes tienen agammaglobulinemia, lo que provoca infecciones recurrentes. La leucemia linfocítica crónica se detecta principalmente de forma incidental en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria análisis de sangre que muestran linfocitosis. Los LOS Neisseria frotis periféricos muestran células de mancha. La leucemia linfocítica crónica es una de las principales causas secundarias de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune tanto por anticuerpos calientes como por anticuerpos fríos y debe considerarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria casos de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune asociados a esplenomegalia masiva o linfadenopatía.
  • Crioglobulinemia: trastorno debido a las inmunoglobulinas (crioglobulinas) que precipitan a bajas temperaturas (por debajo de 37℃). La crioglobulinemia se asocia a artralgias, glomerulonefritis, púrpura o neuropatía periférica. Los LOS Neisseria pacientes también pueden presentar el fenómeno de Raynaud inducido por el frío. Los LOS Neisseria antecedentes de infección (generalmente el virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology de la hepatitis C Hepatitis C Hepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis C Virus) se asocian a la crioglobulinemia. Los LOS Neisseria pacientes no tendrán aglutinación por frío en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las pruebas de laboratorio, y la mayoría no tiene anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoinmune.

Referencias

  1. Brugnara, C., Barcellini,W. (2021). Paroxysmal cold hemoglobinuria. UpToDate. Retrieved April 19, 2021, from https://www.uptodate.com/contents/paroxysmal-cold-hemoglobinuria
  2. Brugnara, C., Brodsky, R.A. (2021). Warm autoimmune hemolytic anemia (AIHA) in adults. UpToDate. Retrieved April 14, 2021, from https://www.uptodate.com/contents/warm-autoimmune-hemolytic-anemia-aiha-in-adults
  3. Luzzatto, L. (2018). Hemolytic anemias. Jameson, J., Fauci A.S., Kasper D.L., Hauser S.L., Longo D.L., Loscalzo J. (Eds.), Harrison’s Principles of Internal Medicine, 20th ed. New York: McGraw-Hill.
  4. Ware, R.E., Despotovic, J.M. (2021). Autoimmune hemolytic anemia in children: classification, clinical features, and diagnosis. UpToDate. Retrieved April 14, 2021, from https://www.uptodate.com/contents/autoimmune-hemolytic-anemia-in-children-classification-clinical-features-and-diagnosis
  5. Ware, R.E., Despotovic, J.M. (2021). Autoimmune hemolytic anemia in children: Treatment and outcome. UpToDate. Retrieved April 14, 2021, from https://www.uptodate.com/contents/autoimmune-hemolytic-anemia-in-children-treatment-and-outcome
  6. Schick, P. (2019). Hemolytic anemia. Medscape. Retrieved April 14, 2021, from https://reference.medscape.com/article/201066-overview

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