Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
25 Cards in this Set
- Front
- Back
|
what is the new term for primary osteomyelofibrosis?
|
agnogenic myeloid metaplasia (AMM)
|
|
|
what are the essentials of diagnosis in myelofibrosis?
|
striking splenomegaly
teardrop poikilocytosis leukoerythroblastic blood picture; giant abnormal plateletes hypercellular bone marrow with fibrosis |
|
|
age distribution in AMM?
|
one fifth under 55 years
in children in first 3 years of life |
|
|
main causes of mortalitiy in AMM?
|
infection
hemorrhage cardiac failure postsplenectomy complications transformation to acute leukemia |
|
|
survival rate in AMM?
|
approximately half that expected for age- and sex-matched controls
|
|
|
10 year survival rate in AMM?
|
<20%
|
|
|
M:F in AMM?
|
M=F, but in children girls affected twice as frequently
|
|
|
proportion of asymptomatic patients in AMM?
|
one fourth
|
|
|
phases in AMM?
|
hyperproliferative early phase with thrombocytosis and leukocytosis
late phase with osteomyelofibrosis |
|
|
differential diagnosis to AMM?
|
secondary myelofibrosis in other MPN (CML, PV)
secondary myelofibrosis due to metastasis of carcinoma hairy cell leukemia, MDS |
|
|
diagnosis of AMM?
|
bone marrow aspirate unsuccessful in 50% --> dry tap
bone marrow biopsy obligatory! |
|
|
therapy principles in AMM?
|
mainly supportive, treatment does not confer prolonged survival
cytoreductive therapy to control leukocytosis, thrombocytosis and organomegaly |
|
|
essentials of AMM?
|
clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells
proliferation primarily of megakaryocytic and granulocytic hematopoietic cells progressive bone marrow fibrosis with extramedullary hematopoiesis |
|
|
chemotherapy in AMM?
|
as means to cytoreductive therapy
hydroxyurea (deoxynucleotide synthesis inhibitor) preferred agent, other agents are interferon-alpha and cladribine (antimetabolite) |
|
|
indication for radiation therapy in AMM?
|
symptomatic extramedullary hematopoiesis
splenic irradiation if splenectomy contraindicated |
|
|
treatment of severe anemia in AMM?
|
androgens and corticosteroids
|
|
|
antiangiogenic treatment in AMM?
|
antiangiogenic and immunmodulatory
thalidomid, lenalidomid |
|
|
prognosis in AMM?
|
worst of all MPN
median survival 3.5-5.5 years |
|
|
risk factors for transformation in AML?
|
leukocytosis and abnormal caryotype
|
|
|
complications of AMM?
|
spleen:
portal hypertension in 7% splenic infarction bone: osteosclerosis, hypertrophic osteoarthropathy and periostitis |
|
|
what are the characteristic laboratory findings in AMM?
|
anemia
white blood count is variable, may be increased to 50'000 platelet count is variable |
|
|
peripheral blood smear in AMM?
|
red cell line with significant poikilocytosis and dakryocytosis and nucleated red blood cells
platelet morphology with megakaryocytic fragments shift of myeloid series with immature forms |
|
|
what is the morphology of the bone marrow in AMM?
|
usually cannot be aspirated (dry tap), early in the course hypercellular with marked increase in megakaryocytes
biopsy with fibrosis demonstrated by silver stain |
|
|
what are the differential diagnoses of a leukoerythroblastic blood picture?
|
response to severe infection, inflammation or infiltrative bone marrow processes
NB teardrop poikilocytosis and giant abnormal plateletes will not be seen |
|
|
what are the differential diagnoses fo bone marrow fibrosis
|
metastatic carcinoma, Hodgkin disease and hairy cell leukemia
|
