Enfermedades mieloproliferativas Flashcards

Question

¿Que resultados se consideran diagnosticos para trombocitosis esencial?

Answer 1

Plaquetas >450,000 /mm3 | No tiene características de otros desordenes mieloproliferativos

Answer 2

``` RBC: No hay WBC: no hay Plaquetas: altas Cromosoma de filadelfia: negativo Mutación JAK2: positivo en 30-50% de casos ```

Answer 3

Minoría desarrolla mielofibrosis | Rara vez se crea leucemia mieloide aguda

Answer 4

JAK2 (50% de casos) | MPL (5-10% de casos)

Answer 5

Poco común | Se ve en adultos mayores

Answer 6

- Hay obliteración de la medula osea con fibrosis debido a mayor actividad de fibroblastos - Asociado a esplenomegalia masiva y RBC de gota

Answer 7

-- Antes de ser una mielofibrosis puede asociarse a leucocitosis y trombosis en sangre periférica e hipercelularidad medular por mayor numero de precursores granulocíticos y megacariocíticos -- Mientras se desarrolla o progresa la enfermedad la medula se reemplaza por matriz fibrosa depositada por fibroblastos reactivos (son estimulados por PDGF y tumor derived growth factor) Los fibrosis distorsiona y borra espacio medular causando que la hematopoyesis pase de medula osea a lugares extramedulares como hígado o bazo

Answer 8

Los fibrosis distorsiona y borra espacio medular causando que la hematopoyesis pase de medula osea a lugares extramedulares como hígado o bazo

Answer 9

``` Anemia Leucoeritroblastosis Plaquetas Leucocitos Esplenomegalia ```

Answer 10

es variable ⅓ presenta trombocitopenia ⅓ se presenta trombocitopénicos

Answer 11

La hematopoyesis

Answer 12

``` RBC: bajo WBC: variable Plaquetas: variable Cromosoma de filadelfia: negativo Mutación JAK2: positivo en 30-50% de casos ```

Answer 13

Presentación clínica | Biopsia de medula osea

Answer 14

Primary myelo brosis is an uncommon disease of older adults. e diag- nosis is based on the characteristic clinical picture and bone marrow biopsy, which is required to demonstrate the marrow brosis (Fig. 20-7). e distortion of the marrow causes progenitors to “herniate” into dilated sinusoids and be released into the peripheral blood, o en contributing to leukoerythroblasto- sis (Chapter 18). Megakaryocytes are usually increased in number and present in abnormal clusters. Late-stage primary myelo brosis is o en associated with massive splenomegaly due to extramedullary hematopoiesis. In some patients, the brotic marrow is converted to bone (osteosclerosis), whereas a minor- ity of others transform to acute myeloid leukemia, which may appear rst in extramedullary sites such as lymph nodes. e overall clinicopathologic picture is identical to that seen when myelo brosis arises out of PCV, essential throm- bocytosis, or CML, suggesting that primary myelo brosis may not be a unique entity but rather a common pathway of progression among di erent myelopro- liferative disorders. Thee prognosis varies depending on the presentation. Patients with leukopenia (white cell count <4000/mm3) or marked leukocytosis (white cell count >30,000/mm3) together with anemia (hemoglobin <10 g/dL) have a median survival time of under a year, whereas those without any of these ndings survive 5 to 8 years on average. Treatment options are limited. Splenectomy is sometimes performed to relieve symptoms related to massive splenomegaly (pain, early satiety, weight loss) but at the risk of eliminating an important source of blood cells and increasing the platelet count with the threat of thromboembolism. Bone marrow transplantation is an option for younger patients. JAK2 inhibitors are being evaluated as a poten- tial targeted therapy in those with disease associated with JAK2 mutations.

Answer 15

- Transformación a una leucemia aguda | - Progresión a mielofibrosis