Flashcards in WBCs And Lymph Nodes Deck (11):
What is ineffective in myelodysplastic syndrome?
Maturation defects associated with ineffective hematopoiesis seen in people 60+. Can be idiopathic or therapy related MDS (tMDS) from prior chemo.
What is seen in the peripheral smear and bone marrow of MDS?
Pelger-Huet (bilobed neutrophils)
Ringed sideroblasts and megalobastoid cells (BM)
Pawn ball megakaryocyte
<20% myoblasts in BM
What are the common translocations seen in AML?
t(15;17) in M3
inv 16 in M4
t(8;21) in M2
AML following MDS
What are the features of M3 (AML)?
Ages 15-59; young male adults
Bleeding tendencies; risk of DIC
t(15:17) fusion gene of RAR-alpha and PML
Responds to all trans retinoic acid
What are the features of M5 (AML)?
Features of CML
Adults 25-60 years
Philadelphia chromosome (ABL and BCR)
WBC more than 100,000
Platelets initially high followed by thrombocytopenia
Hyper cellular bone marrow
Low LAP score
Treat with imatinib mesylate
Features of polycythemia vera.
Neoplasm of multipotent myeloid stem cell
Features of primary myelofibrosis.
Obliterative marrow fibrosis, suppression of hematopoiesis, extra medullary hematopoiesis
Extensive deposition of collagen and reticulin
Neoplastic megakaryocytes release PDGF SND TGFbeta
Tear drop RBC (dacrocyte)
Fatigue, night sweats, anemia, splenomegaly
Features of essential thrombocytosis.
Clonal hematopoietic stem cell disorder
JAK2 mutation - proliferation of platelets
Basophillia, thrombocytosis, and leukocytosis
Abnormal bleeding time
Portal and hepatic vein thrombosis
Erythromyalgia: intense burning or throbbing pain of hands and feet induce by exercise
Langerhans cell histiocytosis.
Proliferation of immature langerhans cells
Birbeck granules on EM (racquet shaped structures)
Multifocal multisystem, Hand-Schuller, eosinophilic granuloma