WBCs And Lymph Nodes

Question 0

What is seen in the peripheral smear and bone marrow of MDS?

Answer 0

Pancytopenia
Hypogranular granulocytes
Pelger-Huet (bilobed neutrophils)
Ringed sideroblasts and megalobastoid cells (BM)
Pawn ball megakaryocyte 
<20% myoblasts in BM

Question 1

What is ineffective in myelodysplastic syndrome?

Answer 1

Maturation defects associated with ineffective hematopoiesis seen in people 60+. Can be idiopathic or therapy related MDS (tMDS) from prior chemo.

Question 2

What are the common translocations seen in AML?

Answer 2

t(15;17) in M3
inv 16 in M4
t(8;21) in M2
AML following MDS

Question 3

What are the features of M3 (AML)?

Answer 3

Ages 15-59; young male adults 
Auer rods
Bleeding tendencies; risk of DIC
t(15:17) fusion gene of RAR-alpha and PML
FLT3 mutation 
Responds to all trans retinoic acid

Question 4

What are the features of M5 (AML)?

Answer 4

Non-specific esterase
Any age
Gum hypertrophy 
Bleeding tendencies
CNS involvement

Question 5

Features of CML

Answer 5

Adults 25-60 years
Myeloproliferative disorder
Philadelphia chromosome (ABL and BCR)
WBC more than 100,000
Platelets initially high followed by thrombocytopenia 
Anemia, basophillia 
Hyper cellular bone marrow
Massive splenomegaly 
Low LAP score
Treat with imatinib mesylate

Question 6

Features of polycythemia vera.

Answer 6

Neoplasm of multipotent myeloid stem cell
Myeloproliferative disorder 
JAK2 mutation
Decreased erythropoietin 
Hyoercellular BM
Hyperuricemia (gout)
Increased viscosity 
DVT

Question 7

Features of primary myelofibrosis.

Answer 7

Myeloproliferative disorder
>50 years
Obliterative marrow fibrosis, suppression of hematopoiesis, extra medullary hematopoiesis
JAK2 mutation
Extensive deposition of collagen and reticulin
Neoplastic megakaryocytes release PDGF SND TGFbeta
Tear drop RBC (dacrocyte)
Nucleated RBC
Hyperuricemia
Fatigue, night sweats, anemia, splenomegaly

Question 8

Features of essential thrombocytosis.

Answer 8

Clonal hematopoietic stem cell disorder
JAK2 mutation - proliferation of platelets
Basophillia, thrombocytosis, and leukocytosis
Abnormal bleeding time
DVT
Portal and hepatic vein thrombosis
Erythromyalgia: intense burning or throbbing pain of hands and feet induce by exercise

Question 9

Langerhans cell histiocytosis.

Answer 9

Proliferation of immature langerhans cells
Birbeck granules on EM (racquet shaped structures)
Multifocal multisystem, Hand-Schuller, eosinophilic granuloma

Question 10

Common translocation in follicular lymphoma.

Answer 10

14;18 - BCL2

CD19, 20, 10

Can transform into diffuse large B cell lymphoma