Histiocitosis de Células de Langerhans

Descripción General

Definición

La histiocitosis de células de Langerhans ( HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia) es un trastorno neoplásico clonal poco frecuente de las células dendríticas (células de Langerhans), que intervienen 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 presentación de antígenos a las células T.

Epidemiología

• Trastorno histiocítico más frecuente
• Incidencia estimada:
  • Niños (< 15 años de edad):
    • 5-8 casos por millón y año
    • Más frecuente 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 1-3 años
  • Adultos: 1-2 casos por millón y año
• Más frecuente 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 varones 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 mujeres
• La incidencia es mayor 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 población blanca.
• Fumar 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 exceso aumenta el riesgo de HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar monosistémica.

Clasificación

Tanto la Histiocyte Society como la OMS disponen de clasificaciones para los LOS Neisseria trastornos histiocíticos.

• La clasificación de la Histiocyte Society incluye trastornos malignos y no malignos.
• La clasificación de la OMS solo aborda los LOS Neisseria trastornos malignos.

Clasificación de la Histiocyte Society

La Histiocyte Society divide los LOS Neisseria trastornos histiocíticos 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 5 categorías 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 lo siguiente:

• Características clínicas
• Características histológicas
• Características inmunofenotípicas
• Características moleculares

Etiología y Fisiopatología

Etiología

La HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia está causada por mutaciones somáticas de genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure que regulan la vía de señalización MAPK/ERK, como:

• BRAF
• MAP2K1
• RAS RAS Renal artery stenosis (RAS) is the narrowing of one or both renal arteries, usually caused by atherosclerotic disease or by fibromuscular dysplasia. If the stenosis is severe enough, the stenosis causes decreased renal blood flow, which activates the renin-angiotensin-aldosterone system (RAAS) and leads to renovascular hypertension (RVH). Renal Artery Stenosis
• ARAF

Fisiopatología

• Mutaciones genéticas → activación de la vía MAPK → expansión clonal de precursores mieloides.
• Las células dendríticas proliferan y se acumulan debido a la proliferación clonal impulsada por la vía MAPK.
• A continuación, las células dendríticas que proliferan anormalmente se infiltran 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 órgano → manifestaciones clínicas

Presentación Clínica

Sitios implicados

La HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia puede presentarse como un trastorno monosistémico o multisistémico.

LCH monosistémica:

• Los LOS Neisseria síntomas sistémicos suelen estar ausentes.
• Los LOS Neisseria órganos afectados pueden presentar una afectación unifocal Unifocal Retinoblastoma o multifocal Multifocal Retinoblastoma.
• A menudo incluye:
  • Hueso
  • Piel
  • Pulmones
  • Hipófisis
  • SNC
  • Ganglios linfáticos

HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia multisistémica:

• Afecta a ≥ 2 órganos
• La combinación de órganos implicados varía, pero puede incluir:
  • Hueso
  • Piel
  • Hipotálamo-hipofisario
  • Ganglios linfáticos
  • Pulmones
  • SNC
  • Hígado
  • Bazo
  • Mucosa oral
• 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 menores de 3 años, la HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia suele ser una enfermedad multisistémica.

HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar

• Enfermedad pulmonar histiocítica más frecuente
• Anteriormente conocida como granuloma eosinofílico de pulmón
• 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
• Asociada al AL Amyloidosis tabaquismo (el 90% de los LOS Neisseria pacientes son ex fumadores o fumadores actuales)
• Se evidencian síntomas constitucionales (por ejemplo, fiebre, pérdida de peso) 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 20% de los LOS Neisseria casos.

HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia no pulmonar

Los LOS Neisseria signos y síntomas dependen del número y la localización de las zonas afectadas.

Complicaciones

Las complicaciones dependen del sistema orgánico afectado, pero pueden incluir:

• Neumotórax
• Enfermedad vascular pulmonar
• Disfunción pulmonar crónica
• Fractura ósea patológica
• Cirrosis hepática
• Perforación intestinal
• Hemorragia digestiva
• Neoplasias malignas secundarias como la leucemia linfoblástica aguda
• Ceguera (poco frecuente)

Diagnóstico

Estudios de laboratorio

Los LOS Neisseria estudios de laboratorio suelen mostrar hallazgos coherentes con los LOS Neisseria sistemas orgánicos implicados y ayudan a descartar otros diagnósticos (la lista no es exhaustiva):

• Exámenes generales:
  • Hemograma completo (con diferencial) → evaluar la afectación de la médula ósea:
    • 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
    • Trombocitopenia
    • Leucopenia
  • Tiempo de protrombina (TP) y tiempo de tromboplastina parcial activado (TTPa) → coagulopatía (especialmente con afectación hepática).
  • Electrolitos → pueden demostrar anomalías relacionadas con trastornos endocrinos.
  • Pruebas de función renal → evaluar la afectación renal.
  • Perfil hepático → ↑ con afectación hepatobiliar.
• Una evaluación endocrina detallada puede incluir:
  • Hormona estimulante de la tiroides (TSH), T4 T4 The major hormone derived from the thyroid gland. Thyroxine is synthesized via the iodination of tyrosines (monoiodotyrosine) and the coupling of iodotyrosines (diiodotyrosine) in the thyroglobulin. Thyroxine is released from thyroglobulin by proteolysis and secreted into the blood. Thyroxine is peripherally deiodinated to form triiodothyronine which exerts a broad spectrum of stimulatory effects on cell metabolism. Thyroid Hormones libre
  • Prolactina y factor de crecimiento similar a la insulina 1 (IGF-1)
  • Cortisol Cortisol Glucocorticoids sérico, hormona adrenocorticotrópica (ACTH)
  • Osmolalidad sérica y urinaria
  • Hormona foliculoestimulante ( FSH FSH A major gonadotropin secreted by the adenohypophysis. Follicle-stimulating hormone stimulates gametogenesis and the supporting cells such as the ovarian granulosa cells, the testicular sertoli cells, and leydig cells. Fsh consists of two noncovalently linked subunits, alpha and beta. Within a species, the alpha subunit is common in the three pituitary glycoprotein hormones (TSH, LH, and FSH), but the beta subunit is unique and confers its biological specificity. Menstrual Cycle) y hormona luteinizante ( LH LH A major gonadotropin secreted by the adenohypophysis. Luteinizing hormone regulates steroid production by the interstitial cells of the testis and the ovary. The preovulatory luteinizing hormone surge in females induces ovulation, and subsequent luteinization of the follicle. Luteinizing hormone consists of two noncovalently linked subunits, alpha and beta. Within a species, the alpha subunit is common in the three pituitary glycoprotein hormones (TSH, LH, and FSH), but the beta subunit is unique and confers its biological specificity. Menstrual Cycle)
  • Testosterona (hombres)
  • Estradiol Estradiol The 17-beta-isomer of estradiol, an aromatized C18 steroid with hydroxyl group at 3-beta- and 17-beta-position. Estradiol-17-beta is the most potent form of mammalian estrogenic steroids. Noncontraceptive Estrogen and Progestins (mujeres)

Imagenología y estudios auxiliares adicionales

• Radiografía basal 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 presentación inicial:
  • Radiografía de tórax → imagen inicial para síntomas pulmonares:
    • Nódulos mal definidos o estrellados
    • Patrón reticulonodular bilateral difuso (zonas media y superior)
    • Quistes o patrón 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 panal 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 zona superior
    • Volumen pulmonar conservado
    • Conservación del ángulo costofrénico
  • Radiografía del hueso/articulación afectado: Las lesiones suelen ser osteolíticas (aspecto “perforado”).
• PET-TC de cuerpo entero con fluorodesoxiglucosa (FDG):
  • La HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia es ávida de FDG, por lo que la PET-TC detecta la afectación orgánica.
  • Superior a la radiografía, la TC o la RM 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 detección de lesiones óseas de HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia
  • Realizada 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 pacientes ≥ 2 años de edad.
  • Recomendada para el estadiaje
• Otros estudios dependen de los LOS Neisseria lugares afectados:
  • RM cerebral con contraste de gadolinio:
    • Para afectación del SNC
    • Detecta lesiones de la masa craneal
    • Útil para evaluar la hipófisis
  • TC:
    • Puede evaluar la afectación gastrointestinal, hepática, esplénica, mediastínica y pulmonar.
    • 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 HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar se observan opacidades reticulonodulares y quistes (zonas media y superior).
  • Pruebas de función pulmonar:
    • Evaluan la afectación pulmonar
    • Pueden ser normales 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 enfermedad temprana
    • Pueden mostrar una capacidad de difusión reducida con anomalías obstructivas, restrictivas o mixtas.
  • Ultrasonido: puede utilizarse 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 evaluación de hígado/tiroides.

Patología

• Examen de la médula ósea (especialmente con un hemograma anormal)
• Biopsia de la lesión/ tumor Tumor Inflammation recomendada 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 todos los LOS Neisseria casos:
  • Ejemplos:
    • Lesión ósea osteolítica
    • Lesión cutánea
    • Lavado broncoalveolar
    • Biopsia de pulmón
  • Las características histopatológicas varían 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 función del lugar de la biopsia, mostrando tejido:
    • Células de Langerhans (núcleos plegados o estriados, con citoplasma moderadamente abundante)
    • Células inflamatorias circundantes (por ejemplo, eosinófilos)
    • Diversos grados de fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans
    • Atipia citológica 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 células neoplásicas
  • La inmunohistoquímica → puede ser positiva para:
    • CD1a
    • CD207 (langerina)
    • S100 S100 A family of highly acidic calcium-binding proteins found in large concentration in the brain and believed to be glial in origin. They are also found in other organs in the body. They have in common the ef-hand motif (ef hand motifs) found on a number of calcium binding proteins. The name of this family derives from the property of being soluble in a 100% saturated ammonium sulfate solution. Acoustic Neuroma
  • Genética:
    • Prueba de mutación BRAF V600E (positiva 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 > 50%)
    • Considerar la secuenciación de nueva generación para la vía de señalización MAPK-ERK

Tratamiento y Pronóstico

La elección del tratamiento de la HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia 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 el tipo de HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia, la presentación clínica, los LOS Neisseria órganos afectados, la extensión de la afectación y la función orgánica. El tratamiento suele estar dirigido por un oncólogo (y potencialmente por otros especialistas).

Tratamiento de la HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar

• Tratamiento de soporte:
  • Cese de tabaquismo
  • Evaluación periódica de la función pulmonar
  • Broncodilatadores inhalados y/o glucocorticoides para la limitación reversible del flujo aéreo
  • Rehabilitación pulmonar 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 pacientes con disnea de esfuerzo
  • Vacunación antineumocócica y de la influenza Influenza Influenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/Influenza estacional
• Función pulmonar levemente sintomática/deteriorada: observación
• HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar sintomática/progresiva:
  • Glucocorticoides sistémicos
  • Opciones de quimioterapia:
    • Cladribina
    • Citarabina

Tratamiento de la HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia no pulmonar

Abordaje:

• Factores que afectan el manejo:
  • Afectación del SNC
  • Otros órganos implicados
  • Enfermedad monosistémica o multisistémica
  • Afectación unifocal Unifocal Retinoblastoma o multifocal Multifocal Retinoblastoma
  • Presencia y grado de los LOS Neisseria síntomas
• Principios generales:
  • Unifocal Unifocal Retinoblastoma, un solo sistema sin afectación de órganos críticos (SNC, corazón, hígado, bazo, médula ósea):
    • Terapia local
    • Observación
  • Multifocal Multifocal Retinoblastoma, multisistémica o unifocal Unifocal Retinoblastoma con afectación de órganos críticos:
    • Terapia sistémica
    • Si está asintomático o sin disfunción orgánica, puede considerarse la observación.

Afectación multisistémica 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:

• Vinblastina
• Prednisona

Afectación multisistémica 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:

• Opciones de quimioterapia:
  • citarabina
  • cladribina
• Terapia dirigida para mutaciones genéticas conocidas:
  • Inhibidores de BRAF (por ejemplo, vemurafenib Vemurafenib An indole sulfonamide compound and inhibitor of BRAF kinases that is used for the treatment of unresectable or metastatic melanoma. Targeted and Other Nontraditional Antineoplastic Therapy o dabrafenib DaBRAFenib Targeted and Other Nontraditional Antineoplastic Therapy)
  • Inhibidores de MEK (por ejemplo, cobimetinib Cobimetinib Targeted and Other Nontraditional Antineoplastic Therapy)

Afectación monosistemica:

• Lesión ósea única (riesgo no de SNC):
  • Curetaje
  • Radioterapia:
    • Para una lesión recurrente o persistente
    • No se utiliza 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
  • El tratamiento con bifosfonatos puede reducir el dolor Dolor Inflammation.
• Lesiones óseas múltiples:
  • Terapia sistémica (quimioterapia y terapias dirigidas similares a las utilizadas para la afectación multisistémica)
  • Tratamientos complementarios:
    • Cirugía
    • Radioterapia
• Dermatológicos:
  • La HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia solo cutánea puede remitir espontáneamente o evolucionar a HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia multisistémica.
  • Pacientes asintomáticos: observación
  • Sintomática o con afectación cutánea extensa:
    • Esteroides tópicos o mostaza nitrogenada
    • Metotrexato, que puede combinarse con otros agentes inmunosupresores (por ejemplo, prednisolona, 6-mercaptopurina, hidroxiurea).
    • Lenalidomida
    • Talidomida

Pronóstico

• La evolución clínica y el pronóstico de los LOS Neisseria pacientes con HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia son variables:
  • Se asocia a un buen pronóstico:
    • Enfermedad unifocal Unifocal Retinoblastoma o monosistémica
    • Afectación de un único ganglio o enfermedad cutánea aislada
    • Ausencia de lesiones 1 año después del seguimiento
    • HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia pulmonar (sin tabaquismo)
  • Se asocia a un peor pronóstico:
    • Enfermedad multifocal Multifocal Retinoblastoma o pacientes de alto riesgo
    • HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia con mutaciones BRAF (mayor probabilidad de recaída)
    • Afectación de órganos de alto riesgo (por ejemplo, SNC, médula ósea, hígado, bazo)
• La HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia focal crónica puede evolucionar a enfermedad multifocal Multifocal Retinoblastoma o diseminada.

Diagnóstico Diferencial

• Mieloma múltiple: neoplasia 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 que las células plasmáticas proliferan de forma anormal 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, provocando el desplazamiento de las líneas celulares hematopoyéticas. La presentación clínica puede incluir dolor Dolor Inflammation óseo, fracturas patológicas y signos de insuficiencia renal. Las lesiones óseas osteolíticas pueden verse 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 imágenes. Las características diagnósticas distintivas son la proteína monoclonal 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 suero y orina, la histología (por ejemplo, células plasmáticas con abundante citoplasma basófilo) y el inmunofenotipo. El tratamiento incluye corticosteroides, quimioterapia y/o agentes inmunomoduladores.
• Enfermedad de Erdheim-Chester: a menudo una enfermedad del adulto, esta enfermedad es un trastorno histiocítico multisistémico. Los LOS Neisseria infiltrados xantogranulomatosos afectan a ≥ 1 sistema orgánico (por ejemplo, piel, pulmón, hueso, cerebro, hipófisis, retroperitoneo, sistema cardiovascular). Se observa osteosclerosis Osteosclerosis An abnormal hardening or increased density of bone tissue. Paget Disease of Bone 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 huesos largos, y el diagnóstico se confirma por las características patológicas, como histiocitos espumosos y células gigantes de Touton (histiocitos multinucleados) 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 un fondo de fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans (CD1a no se expresa).
• Sarcoma de células de Langerhans: neoplasia de alto grado que se presenta con afectación de un solo órgano o multisistémica. La presentación clínica es similar a la de la HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia, pero puede distinguirse por el análisis citológico. El sarcoma de células de Langerhans se caracteriza por una citología maligna (atipia celular y mitosis Mitosis A type of cell nucleus division by means of which the two daughter nuclei normally receive identical complements of the number of chromosomes of the somatic cells of the species. Cell Cycle atípicas).
• Xantogranuloma juvenil: forma más frecuente de HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia no HCL HCL Hairy cell leukemia (HCL) is a rare, chronic, B-cell leukemia characterized by the accumulation of small mature B lymphocytes that have “hair-like projections” visible on microscopy. The abnormal cells accumulate in the peripheral blood, bone marrow (causing fibrosis), and red pulp of the spleen, leading to cytopenias. Hairy Cell Leukemia. El xantogranuloma juvenil afecta predominantemente a niños y se presenta clínicamente con lesiones cutáneas solitarias o múltiples. La dermatoscopia ayuda al AL Amyloidosis diagnóstico. La afección puede resolverse espontáneamente o requerir escisiones cutáneas curativas.
• Enfermedad de Rosai-Dorfman: proliferación anormal y acumulación de histiocitos 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, con rara aparición de afectación extraganglionar. Los LOS Neisseria individuos pueden presentar linfadenopatía (cervical), fiebre, palidez, pérdida de peso, malestar y rinitis crónica. El diagnóstico implica el examen histopatológico (negativo para CD1a y CD207). Puede producirse una remisión espontánea. Los LOS Neisseria tratamientos incluyen terapia de soporte, administración de esteroides o interferón alfa, o quimioterapia.