HEMATOLOGIA BASICA
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Este documento resume los principales temas de hematología general, incluyendo diferentes tipos de anemia, síndromes mielodisplásicos, leucemias, linfomas, trastornos de la coagulación y trombosis. Cubre las causas, síntomas y tratamientos de estas enfermedades hematológicas.
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HEMATOLOGIA BASICA
- 1. Dra. KaMi Apuntes Hematología Hematología general, preguntas y respuestas
- 2. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 3. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 6. Nature Reviews Cancer 5, 543-555 (July 2005)
- 10. epo
- 14. Hb 13.5 Hto 45-55 VGM 80-100 HCM 30-35 ADE ó RDW 12-15 Leucocitos 5000-10 000 Neutrófilos 45-65% ó 1500 tot Linfocitos 35-45% 1500 tot Monocitos 3-10% Eosinófilos Hasta 1000 Basófilos 1-3 % Plaquetas 150-450 mil
- 19. Valores normales VOLUMEN CORPUSCULAR MEDIO 90 ± 8 fL HEMOGLOBINA CORPUSCULAR MEDIA 30 ± 3 pg CONCENTRACION DE HEMOGLOBINA CORPUSCULAR MEDIA 33 ± 2%
- 21. MICROCÍTICA DEFICIENCIA DE FE PLOMO NORMOCÍTICA ENFERMEDAD CRÓNICA ENFERMEDAD RENAL CRÓNICA MACROCÍTICA DEFICIENCIA DE B12 O FOLATO ENFERMEDAD HEPÁTICA HEMÓLISIS
- 43. Cirrosis: grado leve de anemia, deficiencia de lecitin colesterol acil transferasa
- 53. Hb en g/dL HOMBRES <13 MUJERES <12
- 62. N Engl J Med 2004; 350:2383-2397
- 67. RECEPTOR TRANSFERRINA: SE ELEVA Ferritina <20 mcg/L Definitivo <45 mcg/L Muy probable >100 mcg/L Se descarta
- 85. Síntoma Pica Tierra (geofagia), hielo ( pagofagia ) papel o almidón Beturia Betalaina no es oxidada por Fe Síndrome de piernas inquietas Causa, no se sabe la relación Atrofia de papilas gustativas SX PLUMMER-VINSON (PATTERSON KELLY) DISFAGIA, BANDAS DE TEJIDO FIBROSO ESOFÁGICO Y ANEMIA POR DEFICIENCIA DE HIERRO
- 90. Fe Absorber en medio ácido Preguntas sobre en ayunas, el tiempo, el ácido ascórbico…. Dosis Tema favorito de los pediatras: 1.5 – 2 mg/kg de peso en tres tomas, en adultos es de 200 mg al día Duración Recordar que al corregir la anemia se toma de 3 a 6 meses al día (RESERVAS DE FE) Valoración de la respuesta Típicas: primero los retis, después ferritina, la Hb sube a 1g/dL cada 2 semanas Hierro IV No saberse fórmulas, solo saber un gramo de Hb tiene 3.3 mg de Fe
- 102. TERAPIA PUNTOS TRANSFUSIÓN NO TRANSFUNDIR A MENOS QUE LA HB SEA MENOR A 8 g/dL o haya síntomas. Un nivel aceptado universal: no hay (Pbe <6.5) Un paquete sube un gramo Hierro AGUAS: infección y administración de hierro Cuidado hierro y ferritina alta (no se da en gral > 100, en erc niveles más alto, alerta > 800) ERITROPOYETINA No usar en cáncer Si no responde hay una cuasa secundaria Causa subyacente La infección aguda puede bajar 2-3 g/dL en 1 o días por hemolisis. El uso de antiTNF en ar aumenta la hb.
- 113. MECANISMO ENFERMEDAD 1. Hemoglobinopatía estructural Anemia células falciformes Hemoglobinas M 2. Talasemia Defecto en la síntesis de una cadena 3. Variantes talasémicas HbE Hb Lepore Hb primavera constante 4. Persistencia de HbF Hemoglobina fetal en vida adulta 5. Adquiridas Metahemoglobinemia Sulfohemoglobinemia Carboxihemoglobinemia Eritroleucemia
- 124. Hemoglobina Portland ( 2) y Gower I ( 2) Gower ( 2)
- 153. RUTAS INDEPENDIENTES DE DEFICIENCIA VITAMINICA
- 154. MEDICAMENTOS TRIMETROPIM METOTREXATE AZATIOPRINA IMATINIB ZIDOVUDINA
- 159. Absorción Ácido fólico Alimentos folatos Brócoli, espinaca, espárrago, melón, carne, hígado, riñones Duodeno, ID poliglutamatos hidrolizados monoglutamatos Yeyuno-enterocito - citoplasma reducción a FH4 y luego metilación metilFH4 Rc Ác.ascórbico Torrente circulatorio
- 164. ANEMIA MEGALOBLÁSTICAS MACRO OVALOCITOS ANISOCITOSIS POIQUILOCITOSIS CÉLULAS EN DIANA (FALLA HEPÁTICA O ALCOHOLISMO)
- 171. B12 sérica Folato sérico F. eritrocitario 200 - 900pg/ml 5 - 16 ng/ml 150 ng/ml Déficit < 100 N o >16 < 150 de B12 Déficit N < 3 < 150 de folato Déficit < 100 < 3 < 150 de ambos Determinación de niveles vitamínicos
- 198. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 211. CAUSAS TIMOMA LEUCEMIA LINFOCÍTICA CRÓNICA INFECCIÓN POR PARVOVIRUS B19 EMBARAZO MEDICAMENTOS: CLORANFENICOL, AZATIOPRINA, FENITOÍNA
- 219. DEFECTO EN PLAQUETAS DEFECTO EN FACTORES DE LA COAGULACIÓN Lugar del evento hemorrágico Mucocutáneo (epistaxis, gastrointestinal, encías, genitourinario) Sangrado profundo (articulaciones, músculos) Sangrado ante cortadas pequeñas Sí Raro Equimosis Sí Poco común Hamartrosis, hematomas intramusculares Poco común Muy común en deficiencias severas, Sangrado con procedimientos quirúrgicos Generalmente inmediato Se asocia al tipo de procedimiento, grado de deficiencia, puede ser tardado
- 254. PRESENTACIÓN ANEMIA 85% NEUTROPENIA 50% TROMBOCITOPENIA 25%
- 269. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 280. CLAVES FLEBOTOMÍA COMO TRATAMIENTO: META HTO <45% EL PERFIL DE FE CON DEFICIENCIA (SE SOBREUTILIZA) HIDROXIUREA: USAR CUANDO HAY ALTO RIESGO DE TROMBOSIS O SOLO SI LAS PLAQUETAS SON MAYOR A 600,000/mcL. ASA: NO A TODOS PUES AUMENTA RIESGO DE SANGRADO. La dosis debe ser la más baja de 81 mg. TROMBOCITOSIS ESENCIAL: es el mismo, pero en plaquetas. MUTACIÓN DE LA VIA JAK EN EL 95% BUEN PRONÓSTICO EN GENERAL
- 281. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 294. TRANSLOCACIONES t (9;22)= cromosoma filadelfia=bcr-abl=LMC; 25% DE LLA t (15;17)= M3 t (8;21) =M2 inv16= M4Eo
- 297. FACTORES MAL PRONÓSTICO >60 AÑOS CITOGENÉTICA NO FAVORABLES (MONOSOMIA O DEL. CR 5 O 7) POBRE FUNCIONALIDAD MIELODISPLÁSICO
- 302. LLA LMA LLC LMC CLINICA GANGLIOS, HEPATO, ESPLENO LEUCOSTASIS, CID, INFILTRACIÓN ASX, 10-20% SXB 3 FASES CELULAS NO GRANULOS, TdT 95% GRANULOS, MPO POSITIVO INMUNO CD19_B CD3_T CD13 CD 33
- 303. FAB FREC ADULTOS INMUNO PRE CEL B L1,L2 75% TdT, CD19 PRE CEL T L1,L2 20% TdT, CD2, 3, 5, 7, neg CD4, neg CD8 CEL B L3 5% TdT, superf Ig
- 314. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 344. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 362. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 372. Un pool o concentrado plaquetario aumenta en promedio 22,000 plaquetas
- 375. claves Reacción febril no hemolítica Fiebre, más común, no hay hemolisis Reacción hemolítica Fiebre, ictericia y hemosideruria. Si es rápida lo más probable incompatibilidad A,B,Rh Reacción alérgica Rash TRALI Disnea Sobrecarga Disnea más peptido natriurético Infección Sepsis
- 381. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 390. 1. ANEMIA: HIERRO, MEGALOBLÁSTICAS, HEMOLÍTICAS, HEMOGLOBINOPATÍAS 2. ANEMIA APLASICA, MIELODISPLASIA Y SINDROME FALLA MEDULAR 3.POLICITEMIA VERA Y OTRAS MIELOPROLIFERATIVAS 4.LEUCEMIAS MIELOIDES 5.LINFOMAS 6. ENFERMEDADES DE CÉLULAS PLASMÁTICAS 7.TERAPIA TRANSFUSIONAL 8.TRANSPLANTE DE CÉLULAS HEMATOPOETICAS 9.COAGULACIÓN 10. TROMBOSIS VENOSA 11. ANTIPLAQUETARIOS, ANTIFIBRINOLÍTICOS, ANTICOAGULANTES
- 403. Factores de la Coagulación Factor Nombre Factor Duración de la Vida Media I Fibrinógeno 4 a 5 días II Protrombina 3 días III Tromboplastina Tisular IV Calcio V Proacelerina, F. Labil 1 día VII Proconvertina, F. Estable 4 a 6 horas VIII F. Antihemofílico A 12 a 18 horas vW Factor von Willebrand 12 a 18 horas IX F. Antihemofílico B, F. Christmas 18 a 24 horas X Factor Stuart 1 a 2 horas XI Precursor de la tromboplastina plasmática 2 a 3 horas XII Factor Hagemann, F. de contacto 2 horas XIII F. Estabilizante de la fibrina 5 días
Notas do Editor
- Primera parte hematopoiesis: Respuesta correcta es la A. Aunque la anemia pudiera sonar lógico, el mecanismo es indirecto: anemia produce hipoxia y la hipoxia estímula la producción de EPO.
- Los capilares peritubulares tienen células endoteliales que producen la eritropoyetina.
- Concepto importante: La EPO SE PRODUCE EN LAS CÉLULAS DE LOS CAPILARES PERITUBULARES: RECUERDEN LA PARADOJA DEL RIÑON: ÓRGANO MÁS PERFUNDIDO, PERO CON ZONAS DE MUY BAJA OXIGENACIÓN (RAZÓN---CHOQUE---NTA)
- Respuesta correcta: pronormoblasto
- Concepto: hematopoyesis: deriva de celulas con expresión de genes GATA-1 Y FOG-1, SI NO EXISTE EPO SE CONDUCEN DIRECTAMENTE A LA APOPTOSIS
- Valores normales de biometría hemática…… a nivel del mar meno Hb, mujeres menos Hb en relación a los hombre, fumar incrementa Hb en base al número de cigarrillos consumidos.
- CHCM= VCM / HCM
- Este dato es útil, pues habla del recambio que se debe dar de la línea eritroide que generalmente oscila entre 0.8 a 1%
- POLICROMASIA
- Formed mainly by mechanical damage and deformation of particularly fragile erythrocytes. Poikilocytes can be found in pernicious anemia, and many other types of anemia. Neverhless, the type of poikilocytosis is specific for the anemia type - e.g., elongated, pencil shaped cells for iron deficiency anemia, pointed tear-shaped cells for megaloblastic anemias, and myelosclerosis and schizocytes for certain types of haemolytic anemia.
- Los acantocitos se observan en deficiencias de lipoproteínas o enfermedades neurológicas. They can be found in patients with an inherited decrease or complete deficiency of -lipoproteins (abetalipoproteinemia) and in patients with some neurological diseases. They can sometimes be found in azotemia, stomach cancer, and bleeding stomach ulcer.
- Leptocitosis glóbulos rojos hipocrómicos delgados por trastornos como la síntesis de talasemia y deficiencia de hierro graveound most frequently in iron deficiency - sideropenic anemia, as a result of disturbances in the hemoglobin synthesis. They can be found in hypochromic anemia, homozygous and heterozygous forms of C, D, E hemoglobinopathy. The presence of leptocytes in b-thalassemia major is a pathognomonic.
- Erythrocytes of oval or elliptical shape as a result of a defect in the red cell membrane, most likely a disturbance of the structure and constituents of spectrin. Such cells can be found in blood of patients with hereditary elliptocytosis, and sometimes in myelofibrosis. Artefacts are common and should be excluded.
- membrane. Spherocytes have increased thickness (about 3 µm), normal volume, and biconvex shape. Spheroidal cells appear in many types of haemolytic anemia, including hereditary spherocytosis and immune haemolytic anemia, and in burns.
- ALCOHOLISMO O FORMAS RARAS DE ANEMIA HEMOLÍTICA
- Found in patients with congenital hemolytic anemia and megaloblastic anemias. Schizocytes are also formed in hemolytic anemias due to mechanical stress (micro-angiopathy, heart valve prosthesis, severe burns), cancer and myelofibrosis.
- Pear shaped with a more or less extended tail that sometimes ends in a swelling. Significance: They can be found in patient with myelofibrosis
- Present in thick portions of smears, especially in patients with increased erythrocyte sedimentation rate
- Very rare in normal people but common after the removal of the spleen, in some hemolytic anemias and in megaloblastic anemia. They may occur in very severe anemias.
- : Cabot’s rings have been described in pernicious anemia, lead poisoning, leukemia, alcoholic jaundice, and in some forms of severe anemia. They can be seen in patients taking cytostatics
- ¿Si donas sangre como previenes anemia por deficiencia de hierro? Un paquete tiene de 500 cc tiene 250 mg de hierro. Lo ideal y que no causa síntomas es tomar 20 mg de Fe en un donador
- Hiperplasia eritroide
- Derecha: la flecha señala dos sideroblastos normales en un aspirado, izquierda
- Section of a bone marrow aspirate taken from a patient with the anemia of chronic disease. The slide has been stained for iron (Prussian blue reaction) and counterstained with safranin to show nuclear detail. Note the increased iron staining within the voluminous cytoplasm of macrophages (thick black arrows), while there is no staining for iron within the cytoplasm of red blood cell precursors (thin black arrows). This pattern (abundant iron in macrophages and reduced to absent iron in red cell precursors) is quite typical for the anemia of chronic disease, and contrasts with iron deficiency, in which iron is absent from both macrophages and red cell precursors, while normal subjects demonstrate iron in macrophages and red cell precursors.
- Recordar las dosis de hierro de las tabletas
- Pica refers to a perverted appetite for substances not fit as food, such as clay or dirt (geophagia), paper products, or starch (amylophagia). It may be modified by cultural or ethnic factors that affect dietary practices [ 24,25 ]. Pagophagia, or pica for ice, is considered quite specific for the iron deficiency state [ 26,27 ]. It may be present in patients who are not anemic and responds rapidly to treatment with iron, often before any increase is noted in the hemoglobin concentration. In one study of 55 unselected patients with iron deficiency anemia secondary to gastrointestinal blood loss, pica was present in 32 and pagophagia in 28
- En las personas sana se absorbe un 5 a 10% en porciones distales
- If pica is present, it will disappear almost as soon as oral iron therapy is begun, well before there are any changes in the peripheral blood. (See &quot;Causes and diagnosis of anemia due to iron deficiency&quot;, section on 'Pica and pagophagia' .)The patient will note an improved feeling of well-being within the first few days of treatment.In patients with moderate to severe anemia, a modest reticulocytosis will be seen, maximal in approximately 7 to 10 days. Patients with mild anemia may have little or no reticulocytosis.The hemoglobin concentration will rise slowly, usually beginning after about one to two weeks of treatment, and will rise approximately 2 g/dL over the ensuing three weeks. The hemoglobin deficit should be halved by about one month and should return to normal by 6 to 8 weeks
- Algunos datos: 10-20 g
- COOPERATIVIDAD---
- CONCEPTO AFINIDAD
- Fumadores eleva 10-15%
- La hemoglobina fetal tiene mayor unión al oxígeno pues carece del 2,3 bifosfoglicerato
- Red cells from a patient with acquired hemoglobin H disease were incubated in vitro with new methylene blue. Multiple (&quot;golf ball-like&quot;) small inclusions due to precipitation of hemoglobin H are seen as a result of interaction with this dye.
- Hemoglobin E (HbE), a mutation of the beta globin chain, is associated with reduced expression (ie, it is a hemoglobin mutation which also expresses a thalassemic blood picture) and is mildly unstable to oxidative damage [ 20,21 ]. The presence of HbE causes few abnormalities other than microcytosis, target cells, and hypochromia when present in a heterozygous or homozygous state [ 22 ]: Heterozygotes (hemoglobin E trait) are not usually anemic, but may have minimal degrees of microcytosis and hypochromia. Hemoglobin electrophoresis shows approximately 30 percent HbE, 1 percent HbF, and 70 percent HbA. Homozygotes (hemoglobin E disease) have minimal anemia along with hypochromia, target cells, and prominent microcytosis. Hemoglobin electrophoresis shows >90 percent HbE and no Hb A, with the remainder being HbF.
- La hidroxiurea convierte la hemoglobina S a hemoglobina F
- Pappenheimer bodies are iron containing granules in red blood cells that are seen because the iron is aggregated with mitochondria and ribosomes. They appear as faint violet or magenta specks, often in small clusters, due to staining of the associated protein. They are associated with severe anemias and thalassemias.
- iver biopsy sections from a 26-year-old man with autosomal recessive congenital sideroblastic anemia and moderate iron overload.
- Peripheral blood smear from a patient with refractory anemia with excess blasts (RAEB) shows a neutrophil with a bilobed pseudo-Pelger-Huet (Pelgeroid) nucleus. The two lobes are connected by a thin strand (arrow) giving a &quot;pince-nez&quot; appearance. These nuclei look identical to the those seen in the inherited Pelger-Huet anomaly. This neutrophil also has markedly reduced granulation, a finding commonly seen in the myelodysplastic syndromes. From Brunning, RD, McKenna, RW. Tumors of the bone marrow. Atlas of tumor pathology (electronic fascicle), Third series, fascicle 9, 1994, Washington, DC. Armed Forces Institute of Pathology.
- Step 1: One µg of radioactive crystalline B12 is taken orally. Step 2: Gastric acid and pepsin free vitamin B12 from food proteins. This step is not required when crystalline B12 is utilized as the test dose. B12 attaches to &quot;R&quot; binders (R) produced by the stomach, which have a higher affinity for B12 than intrinsic factor (IF), also produced by the stomach. Step 3: Pancreatic proteases degrade the &quot;R&quot; binders, allowing formation of the B12-IF complex, the specific form absorbed by the terminal ileum. Step 4: One hour after the test dose, a 1000 µg &quot;flushing&quot; dose of non-radioactive B12 is given to saturate B12 binders (transcobalamines). Step 5: If present, bacteria in &quot;blind loops&quot; in the duodenum or jejunum preferentially utilize vitamin B12, allowing none to be available at the site of absorption. Step 6: With blood and tissue B12 binding sites blocked by the &quot;flushing&quot; dose, the B12/IF complex is absorbed by the terminal ileum, and the B12 is excreted in the urine (step 7), which is assayed for its content of radioactivity. The test can be repeated with the addition of missing factors (eg, intrinsic factor, pancreatic extract), or following the use of nonabsorbable antibiotics (blind loops and/or bacterial overgrowth present), or gluten-free diet (celiac disease).
- Recordar las dosis de requerimientos (D) y la preventiva (B)
- IgG anti–red cell autoantibodies. A, Structure of an IgG molecule demonstrating its variable and constant regions and the heavy and light chains. B, Agglutination of red cells by pentameric IgM antibodies, which can join the cells into a lattice. C , Coating of red cells by IgG antibodies. The antibodies are unable to agglutinate the cells. D , Agglutination of IgG-coated red cells by an anti-IgG antibody.
- Prednisona en AHAI, Transfusión solo en urgencia, Esplenectomía no primera elección, eculizumab es un anticuerpo utilizado en hemoglobinuria paroxistica nocturna.
- La paciente tiene aplasia pura de serie roja….
- These syndromes include clonal diseases (paroxysmal nocturnal hemoglobinuria, myelodysplasia, and large granular lymphocytosis); and single hematopoietic lineage deficiency diseases (agranuloyctosis, pure red-cell aplasia, and amegakaryocytic thrombocytopenia); note especially the areas of overlap between aplastic anemia and paroxysmal nocturnal hemoglobinuria and myelodysplasia.
- Parvovirus el típico y su célula pronormoblasto gigante
- Pronormoblasto gigante: patognomonico de aplasia ligada a parvo; estandar es la detección de DNA en sagnre pues la serología esta ausente.
- Tratamiento con igg 0.4 g/kg diariamente por 5 días. Pueden recibir inmunosupresores ciclosporina, timo, azatioprina, ciclofosfamida,
- Recordar la asociación con timoma, la b solo en caso de que fuera por def de fe con sospecha de sangrado oculto, la c se refiere a hpn y la d solo si se sospechara toxicidad. La piridostigmina no causa comunmente aplasia pura, sino se relaciona más a eritropoyetina acetilada.
- ANEMIA APLÁSICA
- LA CAUSA MÁS COMÚN ES IDIOPATICA
- Puntos clave: la citometría de flujo detecta cd55 y cd 59 Ojo: la anemia aplásica y al hpn se correlacionan en varias maneras…
- he risk of both the myelodysplastic syndromes and acute myeloid leukemia (AML) is increased in certain genetic syndromes: the Diamond–Blackfan syndrome (pure red-cell hypoplasia with craniofacial, skeletal, or cardiac defects), the Shwachman–Diamond syndrome (neutropenia, exocrine pancreatic insufficiency, and short stature), dyskeratosis congenita (anemia and thrombocytopenia with cutaneous pigmentation, nail dystrophy, and leukoplakia), Fanconi's anemia (aplastic anemia with short stature and other skeletal abnormalities), and severe congenital neutropenia. 4 In contrast, there is little information on hereditary predispositions for nonsyndromic forms of the disease, with the exception of a familial platelet disorder associated with a monoallelic germ-line mutation in RUNX1, the gene encoding runt-related transcription factor 1 on chromosome 21q22. This genomic region is frequently involved in chromosomal translocations and somatic point mutations in the acute leukemias, sporadic myelodysplastic syndromes, myelodysplastic syndrome with myeloproliferative features, and therapy-related myeloid neoplasms. 5
- Panel A shows a peripheral-blood sample from a patient with refractory anemia with ring sideroblasts, with dimorphic red cells; some of the cells are normochromic whereas others are hypochromic (arrow). There is also anisocytosis with occasional macroovalocytes (arrowhead).
- Panel B shows a peripheral-blood sample from a patient with refractory anemia with excess of blasts, demonstrating pseudo–Pelger–Huet cells with hypercondensed chromatin and hypolobulated nuclei and virtually colorless cytoplasm (arrow).
- Panel C shows dyserythropoiesis (arrows) in a bone marrow sample obtained from a patient with refractory cytopenia with multilineage dysplasia.
- Panel D shows ring sideroblasts (arrows) from a patient with refractory anemia. The ring sideroblasts are characterized by at least five granules of iron that encircle the nucleus of the erythroid precursor.
- Panel E shows numerous dysplastic small megakaryocytes (arrows) with monolobed or bilobed nuclei and mature granular cytoplasm in the aspirate smear of a patient with refractory anemia with excess of blasts.
- Panel F shows a tissue section from bone marrow of a patient with a myelodysplastic syndrome and isolated del(5q). The megakaryocytes are of medium size, with hypolobulated nuclei (arrows).
- Auer rods can be seen in the leukemic blasts of acute myeloid leukemia . Auer rods are clumps of azurophilic granular material that form elongated needles seen in the cytoplasm of leukemic blasts. They are composed of fused lysosomes and contain peroxidase , lysosomal enzymes, and large crystalline inclusions. Auer rods are classically seen in myeloid blasts of M1, M2, M3, and M4 acute leukemias .
- In typical 5q minus syndrome, the commonly deleted region (CDR) has been mapped to 5q33.1 (at right), which contains SPARC, the gene encoding osteonectin (secreted protein, acidic, cysteine-rich), and RPS14, the gene encoding ribosomal protein S14. In the del(5q)-associated myelodysplastic syndrome–acute myeloid leukemia, the commonly deleted region has been mapped to 5q31.2 (at left), which contains genes encoding catenin alpha 1 ( CTNNA1), early growth response 1 (EGR1), and cell division cycle 25 homologue C (CDC25C).
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- Mixed cellularity Hodgkin's disease. A Reed-Sternberg cell is present near the center of the field; a large cell with a bilobed nucleus and prominent nucleoli giving an &quot;owl's eyes&quot; appearance. The majority of the cells are normal lymphocytes, neutrophils, and eosinophils that form a pleiomorphic cellular infiltrate