SEMINARIO Piel y trastornos hematológicos
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Transcript of SEMINARIO Piel y trastornos hematológicos
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Piel y Piel y HematologíHematologí
aa
Sandra Milena Acevedo Sandra Milena Acevedo RuedaRueda
MD Residente Medicina MD Residente Medicina InternaInterna
Marzo de 2014Marzo de 2014
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IntroducciónIntroducción
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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IntroducciónIntroducción
• Cambios en la colocación
• Cambios derivados de la extravasación de plasma
Palidez, ictericia, eritrodermia, cianosis, Palidez, ictericia, eritrodermia, cianosis, hematopoyesis extramedularhematopoyesis extramedular
Hemostasia primaria y purpuraHemostasia primaria y purpura
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Mayani et al, Cancerología 2 (2007): 95-107
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Mayani et al, Cancerología 2 (2007): 95-107
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Mayani et al, Cancerología 2 (2007): 95-107
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ERITROPOYETINA
Mayani et al, Cancerología 2 (2007): 95-107
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Mayani et al, Cancerología 2 (2007): 95-107
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VM GR -120 días y RET-4 días
Bazo:Eliminación de eritrocitos defectuososSecuestro de hematíes normales y plaquetasHematopoyesis extramedularEliminación de microorganismosRegulación circulación portal
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Hoffman. Hematology: Basic Principles And Practice, 5th Ed. Elsevier, 2008
AnemiaAnemia
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-Rango de HB 13.0 to 14.2 g/dL para hombres y 11,6 to 12.3 g/dL para mujeres
-WHO criteria: hombres <13 y mujeres <12 g/dL *
-WHO/National Cancer Institute's criteria: hombres <14 y mujeres <12 g/dL en pacientes con malignidad *
-NHANES III and Scripps-Kaiser: 12.7 g/dL hombres negros menores de 60 años y menor de 11.5 g/dL para mujeres negras menores de 20 años
* WHO Global Database on Anaemia; Worldwide prevalence of anaemia 1993– 2005.
Valores normalesValores normales
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Valores normalesValores normales
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-En médula ósea 3 días y medio-En circulación periférica 1 día-Valor normal 1%-De 100 GR , 1 es reticulocito-CONTEO ABSOLUTO DE RETICULOCITOS - CAR
Valor que me da bacteriologíaConfiar ??
100 -- 1% 60-- 1,6%
ReticulocitosReticulocitos
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CONTEO CORREGIDO DE RETICULOCITOS
Hto Paciente Hto Teorico
X CAR (%)
CCR
INTERPRETACION>2,5 Anemia hemolitica
<2,5 Anemia hipoproliferativa (Vit B12, A. folico, Fe, Medula osea, EPO)
INTERPRETACION>2,5 Anemia hemolitica
<2,5 Anemia hipoproliferativa (Vit B12, A. folico, Fe, Medula osea, EPO)
ReticulocitosReticulocitos
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EL TIEMPO DE SALIDA A LA CIRCULACION PERIFERICA AUMENTA CADA VEZ QUE HAY MAS ANEMIA
EL TIEMPO DE SALIDA A LA CIRCULACION PERIFERICA AUMENTA CADA VEZ QUE HAY MAS ANEMIA
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Y EN ANEMIA SEVERA?
HEMATOCRITO < 25
INDICE DE PRODUCCION DE RETICULOCITOS
CCR 2 IPR
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El oxígeno es liberado de la hemoblogina acorde con la curva de
disociación de la hemoglobina
Cada gramo de HB lleva 1.3 mL de oxígeno
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ALGORITMO ANEMIA
Adapted from Nathan, DG, Oski, FA. Hematology of Infancy and
Childhood, 4th ed, WB Saunders, Philadelphia, PA 1993. p. 352.
Clasificación Clasificación WintrobeWintrobe
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Clasificación BessmanClasificación Bessman
Ayalew Tefferi, Et al. Mayo Clin Proc. 2003;78:1274-1280
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Piel y HematologíaPiel y HematologíaPalidez
Palmas, cara, lecho ungueal, conjuntivasEnfermedades crónicas, medicamentos, frío, shock,
anemia
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Pliegues de las palmas:Pliegues de las palmas:Buen indicador de anemiaBuen indicador de anemia
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
AnemiaAnemia
Síntomas constitucionalesSíntomas constitucionales
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Ferropenia Ferropenia
Pica
Síntomas constitucionales
Causas
TratamientoSkin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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FLUSHINGFLUSHING
Vasodilatación
Sudoración
Fibras colinérgicas
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Policitemia veraPolicitemia vera
Clínica
Acrocianosis rubicundaAcrocianosis rubicundaPlétoraPlétora
Cefalea, parestesias, eritromelalgia, prurito acuógeno
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Síndromes mielodisplasicos
Neoplasias mieloproliferativas• Clásicas (PV, PV, TE, LMC, MP, LNC, LEC,Enf Mast)• Atípicas (LMMC, LMMJ, LMCa, NoClas)
Trastornos Trastornos mieloides crónicosmieloides crónicos
Diagnostic approach to the patient with polycythemia. Ayalew Tefferi, MD, Stanley L Schrier, MD, Stephen A
Landaw, MD, PhD. Nov, 2012
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CianosisCianosis
Clínica
DescoloraciónDescoloraciónPúrpura, azulPúrpura, azul
Periférica: Labios, pabellón auricular, lecho ungueal, manos
Central: Lingual, sublingual
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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ICTERICIAICTERICIA
Clínica
Coloración amarillaColoración amarillaEscleras, sublingual, pielEscleras, sublingual, piel
Hiperbilirrubinemia
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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HEMATOPOYESIS HEMATOPOYESIS EXTRAMEDULAREXTRAMEDULAR
Dermis: Hematopoyesis desarrollo fetal
Pápulas, placas violáceas, ulcerasPápulas, placas violáceas, ulceras
Eritroblastosis fetal, alteraciones cromosómicas, anemia hemolítica congénita, talasemias,
síndrome transfusión feto feto
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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HEMATOPOYESIS HEMATOPOYESIS EXTRAMEDULAREXTRAMEDULAR
Dermis: Hematopoyesis desarrollo fetal
Pápulas, placas violáceas, ulcerasPápulas, placas violáceas, ulceras
Adultos: Mielofibrosis, talasemias, anemias hemolíticas
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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HEMOSTASIA HEMOSTASIA PRIMARIAPRIMARIA
Defectos menores microvasculatura
Vasoconstricción, adhesión, activación
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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PURPURAPURPURA
Hemorragia mucocutanea5 – 10 mm diametro
Palpable / No palpableNo desaparecen
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
Vasculares, hematológicas, Vasculares, hematológicas, reumatológicas, traumáticas, reumatológicas, traumáticas,
infecciosasinfecciosas
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PURPURAPURPURA
Palpable: edema e inflamaciónPrecede isquemia microvascular
Reticular: Vaso-oclusión
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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PTIPTI
Menorragia, epistaxis, sangrado gingival
Ac contra plaqueta Rp GIIB/IIIA y IB
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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PT AloinmunePT Aloinmune
Transfusiones sanguíneas o plaquetasHemorragia maternofetal
CD 109: plaquetas, endotelio, linfocitosT
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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PTTPTT
Adultos: ADAM 13También: CID, PURPURA FULMINANS
Niños: SHU : e. Coli 0157:H7
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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PURPURA PURPURA FULMINANSFULMINANS
•Extensas áreas de necrosis hemorrágica azul-negruzco
•Vasculitis pequeños vasos con microtrombosProteína CProteína C
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
MeningococcemiaMeningococcemiaGram +, Gram -Gram +, Gram -
MeningococcemiaMeningococcemiaGram +, Gram -Gram +, Gram -
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CIDCID
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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LEUCEMIA CUTISLEUCEMIA CUTIS
• Diseminada ó localizada • Enfermedad sistémica
• 5 a 40% leucemias• Recaída
Leucemia monocítica aguda M5, Leucemia mielomonocítica aguda M4
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Pápulas, placas o nódulos (pequeñas), gingivalCara , tronco, extremidades
Eritrodermia
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PRONÓSTICO DEACUERDO AL TIPO DE ENFERMEDAD
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Linfomas cutáneosLinfomas cutáneos
Linfocitos B, TNatural killer
Células dendríticas plasmocitoides
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Linfomas cutáneosLinfomas cutáneos
EtiologíaClon malignoViral ? •HTLV-1•Epstein Barr
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
S. AureusS. AureusExpansión clonalExpansión clonalInfiltración linfocíticaInfiltración linfocíticaEnterotoxinasEnterotoxinas
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Linfomas cutáneosLinfomas cutáneos
Fas, FasL – ApoptósisAlteración a dicho nivel en el linfoma
cutáneo (melanoma)
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Linfomas cutáneos Linfomas cutáneos Células TCélulas T
Micosis fungoidesHombres 2:1
De 50 a 60 años
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Linfomas cutáneos Linfomas cutáneos Células TCélulas T
Micosis fungoides•Placa, parche, tumor•Años de evolución
•Una o varias lesiones
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
Prefieren axilas, Prefieren axilas, ingles, área ingles, área
inframamaria, fosa inframamaria, fosa antecubitalantecubital
Prefieren axilas, Prefieren axilas, ingles, área ingles, área
inframamaria, fosa inframamaria, fosa antecubitalantecubital
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Micosis fungoidesMicosis fungoides
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Variantes de micosis Variantes de micosis fungoidesfungoides
• MF foliculotropa– Mucinosis folicular– Alopecia mucinosa
• MF hipopigmentada• Reticulosis pagetoide• Granulomatosis Slack skin
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Síndrome de SézarySíndrome de Sézary
Definición : Definición : Eritroderma, linfadenopatía, células T nucleo cerebriforme
Manifestaciones clínicas: Manifestaciones clínicas: exfoliación{on, edema, liqueinificación, prurito intenso
Laboratorios : Laboratorios : 1000 celulas, CD4/CD8
Tratamiento y pronóstico: Tratamiento y pronóstico: Pronostico pobre. 33% a 5 años, Infecciones
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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•Papulas o nódulos centro necrótico•Tronco y extremidades
•Metotrexate•Buen pronostico, 5 años, 100%
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
PAPULOSIS PAPULOSIS LINFOMATOIDELINFOMATOIDE
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Linfoma de celulas Linfoma de celulas grandes anaplasicograndes anaplasico
Expresan CD 30, celulas grandes
• Adultos en mayoria• Raro en niños• Nodulos, tumores: rojos, cafés: ulcerados• Radioterapia, PUVA, INF a
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Linfoma Celulas T , Linfoma Celulas T , paniculitis-likepaniculitis-like
• Linfoma cel T citotoxicas• Sindrome hemofagocitico• Piernas Generalmente• Placas, nodulos• Sintomas B
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Piel y HematologíaPiel y Hematología
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LINFOMAS B LINFOMAS B CUTANEOSCUTANEOS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
Linfoma cutáneo zona marginal
Linfoma cutáneo células B grandes de la pierna
Pobre pronosticoSobrevida 14 meses
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Pseudolinfomas Pseudolinfomas cutáneoscutáneos
Hiperplasia linfoide cutánea
Enfermedad de Kimura
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Idiopatica en la mayoríaCuerpos extraños, joyas, trauma, infecciones, acupuntura, tatuajes,
medicamentos
Idiopatica en la mayoríaCuerpos extraños, joyas, trauma, infecciones, acupuntura, tatuajes,
medicamentos
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
HIPERPLASIA LINFOIDE HIPERPLASIA LINFOIDE CUTANEACUTANEA
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CBZBB
FENITOINAFENOBARBITAL
CAIECA
ALOPURINOL CICLOSPORINA
INHIBIDORES H1 H2 HISTAMINA
CBZBB
FENITOINAFENOBARBITAL
CAIECA
ALOPURINOL CICLOSPORINA
INHIBIDORES H1 H2 HISTAMINA
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
HIPERPLASIA LINFOIDE HIPERPLASIA LINFOIDE CUTANEACUTANEA
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Nódulos más de 10 cmCabeza y cuello
Nódulos más de 10 cmCabeza y cuello
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
Asociado a VIH
Asociado a Herpes 8
Asociado a Kapossi
Manifestaciones: POEMS
Variante de celulas plasmáticas adultos mayoresVariante vascular adultos jóvenes
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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HISTIOCITOSIS HISTIOCITOSIS LANGERHANSLANGERHANS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
•Pápulas
•2 mm localizadas
•Agresividad
•Tronco y cuero cabelludo
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HISTIOCITOSIS HISTIOCITOSIS LANGERHANSLANGERHANS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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•Pancitopenia•Mal pronóstico•Muerte cercana
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HISTIOCITOSIS HISTIOCITOSIS LANGERHANSLANGERHANS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Xantogranuloma juvenilHistiocitosis cefálica benigna
Histiocitosis Celulas No Histiocitosis Celulas No LargerhansLargerhans
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GlaucomaGlaucomaCegueraCeguera
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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MASTOCITOSISMASTOCITOSIS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
HuesoHuesoPielPiel
BazoBazoHígadoHígado
TGITGI
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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MASTOCITOSISMASTOCITOSIS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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MASTOCITOSISMASTOCITOSIS
Skin Manifestations of Bone Marrow and Blood Disorders. Sec 25. Fitzpatrick’s Dermatology, 2009
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Niños: Buen pronóstico
Adultos: Peor pronostico, progresiva
MASTOCITOSISMASTOCITOSIS
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