La anemiaAnemiaAnemia 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 anemiaAnemiaAnemia 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 losLOSNeisseria eritrocitos propios, lo que provoca la destrucción de estas células enENErythema 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 losLOSNeisseria autoanticuerpos. La etiología es diversa e incluye infecciones, enfermedades autoinmunes, trastornos linfoproliferativos y medicamentos. El diagnóstico se basa enENErythema 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 (LDHLDHOsteosarcoma y bilirrubina indirecta elevada, haptoglobina reducida) enENErythema 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 enENErythema 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 NodosumlosLOSNeisseria casos refractarios.
La anemiaAnemiaAnemia 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 losLOSNeisseria eritrocitos por autoanticuerpos. Existen dos categorías de anemiaAnemiaAnemia 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 enENErythema 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 losLOSNeisseria autoanticuerpos:
AnemiaAnemiaAnemia 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
AnemiaAnemiaAnemia 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 alALAmyloidosis año
Es ligeramente más probable que se produzca enENErythema 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 enENErythema 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 NodosumlosLOSNeisseria hombres
Causa secundaria presente enENErythema 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 losLOSNeisseria casos reportados
AnemiaAnemiaAnemia 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 enENErythema 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 enENErythema 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
AnemiaAnemiaAnemia 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 enENErythema 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
AnemiaAnemiaAnemia 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 losLOSNeisseria casos)
Trastornos linfoproliferativos
Trastornos autoinmunes
Malignidad
Inmunodeficiencia
Infecciones
Embarazo
Medicamentos
Trasplante alogénico de células madre hematopoyéticas: normalmente entre 2–6 meses después del injerto
AnemiaAnemiaAnemia 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
HemoglobinuriaHemoglobinuriaThe 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 enENErythema 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)
AnemiaAnemiaAnemia 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:
Causas específicas de anemiaAnemiaAnemia 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 anemiaAnemiaAnemia 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 IgGIgGThe major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of igg, for example, igg1, igg2a, and igg2b.Hypersensitivity Pneumonitis)
Trasplante alogénico de células madre hematopoyéticas
Embarazo
Medicamentos
Penicilina
Ceftriaxona
Metildopa
Tabla: Causas específicas de la anemiaAnemiaAnemia 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 IgMIgMA 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
EBVEBVEpstein-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 hemoglobinuriaHemoglobinuriaThe 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 enENErythema 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 NodosumlosLOSNeisseria niños.
Infección bacteriana
Mycoplasma pneumoniaeMycoplasma pneumoniaeShort 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 hemoglobinuriaHemoglobinuriaThe 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 KlebsiellaKlebsiellaKlebsiella 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 coliEscherichia coliThe 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)
AnemiaAnemiaAnemia 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
LosLOSNeisseria autoanticuerpos calientes son responsables de la mayoría de losLOSNeisseria casos de anemiaAnemiaAnemia 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
LosLOSNeisseria anticuerpos (predominantemente IgGIgGThe 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 losLOSNeisseria antígenos del complejo del factor Rhesus o losLOSNeisseria antígenos glicoproteicos.
LosLOSNeisseria autoanticuerpos calientes reaccionan más fuertemente a 37°C.
Patogénesis:
EnENErythema 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 losLOSNeisseria casos, la hemólisis (facilitada por las células fagocíticas) se produce de forma extravascular.
La destrucción de losLOSNeisseria eritrocitos se produce donde losLOSNeisseria macrófagos son abundantes, sobre todo enENErythema 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 enENErythema 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 FcFcCrystallizable 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 receptorReceptorReceptors 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.ReceptorsFcFcCrystallizable 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 losLOSNeisseria fagocitos → fagocitosis de la cadena pesada de la IgGIgGThe 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 enENErythema 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
LosLOSNeisseria macrófagos del hígado reconocen losLOSNeisseria fragmentos del complemento C3 → LosLOSNeisseria eritrocitos con complemento son fagocitados.
Más del 95% de losLOSNeisseria casos de anemiaAnemiaAnemia 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 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.
AnemiaAnemiaAnemia 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
LosLOSNeisseria anticuerpos (predominantemente IgMIgMA 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 enENErythema 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 enENErythema 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 losLOSNeisseria eritrocitos, losLOSNeisseria autoanticuerpos IgMIgMA 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).
LosLOSNeisseria eritrocitos recubiertos por C3b son sometidos a fagocitosis extravascular (enENErythema 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 enENErythema 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 enENErythema 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
AlALAmyloidosis calentarse, la IgMIgMA 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 enENErythema 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 losLOSNeisseria antígenos de losLOSNeisseria eritrocitos (a menudo el antígeno P) enENErythema 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 → hemoglobinuriaHemoglobinuriaThe 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 anemiaAnemiaAnemia 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 enENErythema 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 hemoglobinuriaHemoglobinuriaThe 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
AnemiaAnemiaAnemia 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 (hemoglobinuriaHemoglobinuriaThe 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
AnemiaAnemiaAnemia 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:
Puede provocar gangrena enENErythema 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 reticularisLivedo reticularisA 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
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
El diagnóstico se realiza por evidencia de hemólisis y test de Coombs directo positivo.
Hemograma:
AnemiaAnemiaAnemia 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 losLOSNeisseria eritrocitos
La fuerza de la prueba positiva se correlaciona con la gravedad de la hemólisis.
AnemiaAnemiaAnemia 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.
AnemiaAnemiaAnemia 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
↑ ASTASTEnzymes of the transferase class that catalyze the conversion of l-aspartate and 2-ketoglutarate to oxaloacetate and l-glutamate.Liver Function Tests y ALTALTAn enzyme that catalyzes the conversion of l-alanine and 2-oxoglutarate to pyruvate and l-glutamate.Liver Function Tests
Haptoglobina sérica baja
LosLOSNeisseria niveles de C3 y C4 se reducen enENErythema 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 anemiaAnemiaAnemia 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 enENErythema 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 anemiaAnemiaAnemia 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
Principio de la prueba de Coombs directa: Se añade a la muestra de sangre un reactivo antiinmunoglobulina humana (dirigido contra las inmunoglobulinas, concretamente contra la IgG, y un fragmento del tercer componente del complemento (C3d)). El reactivo anticuerpos se se unen a y aglutinarán los RBCs si ya tienen una superficie IgG y/o C3d.
Imagen por Lecturio.
Pruebas de Coombs directas e indirectas: Prueba de Coombs directa: Anticuerpos anti-humanos anticuerpos se añaden a los eritrocitos lavados del individuo RBCs . Prueba de Coombs indirecta: Donante eritrocitos del donante y anticuerpos antihumanos anticuerpos se añaden al suero del individuo. Resultado positivo: aglutinación de eritrocitos Resultado negativo: no hay aglutinación de eritrocitos
Imagen por Lecturio.
Periférico frotis de sangre periférica : Los esferocitos son pequeños y redondos RBCs causados por una fagocitosis de su membrana por el bazo fagocitos del bazo (inducida por antígenos unidos a la membrana).
Imagen: “Warm autoimmune hemolytic anemia” por Ed Uthman. Licencia: CC BY 2.0
Evaluación adicional
Análisis de orina, con tira reactiva y microscopio:
HemoglobinuriaHemoglobinuriaThe 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 enENErythema 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 ureaUreaA 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 enENErythema 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 pneumoniaeMycoplasma pneumoniaeShort 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 enENErythema 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)
Evaluar la gravedad de la anemiaAnemiaAnemia 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 losLOSNeisseria 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 losLOSNeisseria autoanticuerpos).
AnemiaAnemiaAnemia 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 +/- rituximabRituximabA 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 rituximabRituximabA 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
AnemiaAnemiaAnemia 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 rituximabRituximabA 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., rituximabRituximabA 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 enENErythema 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
AnemiaAnemiaAnemia 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 anemiaAnemiaAnemia 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 anemiaAnemiaAnemia 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 losLOSNeisseria casos responden a la terapia enENErythema 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 losLOSNeisseria pacientes deben ser instruidos para buscar atención médica si losLOSNeisseria síntomas se repiten.
AnemiaAnemiaAnemia 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
EnENErythema 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 hemoglobinuriaHemoglobinuriaThe 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 virusVirusViruses 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 losLOSNeisseria niños, la recuperación se observa enENErythema 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.
LosLOSNeisseria episodios recurrentes son más frecuentes enENErythema 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 NodosumlosLOSNeisseria adultos.
Diagnóstico Diferencial
AnemiaAnemiaAnemia 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 losLOSNeisseria eritrocitos. La hemólisis puede ser intravascular o extravascular (enENErythema 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 losLOSNeisseria eritrocitos, defectos enzimáticos de losLOSNeisseria eritrocitos, destrucción inmunitaria de losLOSNeisseria eritrocitos, lesiones mecánicas e hiperesplenismo. EnENErythema 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 anemiaAnemiaAnemia 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 enENErythema 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 NodosumlosLOSNeisseria 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, serositisSerositisInflammation of a serous membrane.Systemic Lupus Erythematosus, citopenias, enfermedad tromboembólica, convulsiones y/o psicosis. El diagnóstico se realiza mediante el cumplimiento de losLOSNeisseria criterios clínicos, que incluyen anticuerpos antinucleares, anticuerpos específicos del LES y hallazgos clínicos específicos.
Leucemia linfocítica crónica: la leucemia más común enENErythema 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 enENErythema 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 enENErythema 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 NodosumlosLOSNeisseria 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 enENErythema 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 NodosumlosLOSNeisseria análisis de sangre que muestran linfocitosis. LosLOSNeisseria frotis periféricos muestran células de mancha. La leucemia linfocítica crónica es una de las principales causas secundarias de anemiaAnemiaAnemia 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 enENErythema 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 NodosumlosLOSNeisseria casos de anemiaAnemiaAnemia 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. LosLOSNeisseria pacientes también pueden presentar el fenómeno de Raynaud inducido por el frío. LosLOSNeisseria antecedentes de infección (generalmente el virusVirusViruses 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 CHepatitis CHepatitis 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. LosLOSNeisseria pacientes no tendrán aglutinación por frío enENErythema 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 anemiaAnemiaAnemia 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.
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