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Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. What is the purpose of the fibrin mesh
Acts to stabilize platelet plug
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
No antigen - both antibodies
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

2. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
Rhogam - Rh antigen immunoglobulin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
B antigena and A antibodes

3. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Anemia of chronic disease - Aplastic anemia - kidney disease
Hereditary spherocytosis - autoimmune hemolysis
Blasts > 5%

4. What substance accumulates in porphyria cutanea
CML to AML or All
M3 AML (acute promyelocytic leukemia)
Protein C or S deficiency
Uroporphyrin (tea colored urine)

5. Which factor does vWF carry/protect
120 days
VIII
Beta 4
Beta chain underproduced - asymptomatic - heterozygote

6. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
No lytic bone lesions in WM
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
All

7. Who has more severe disease - HbSS or HbSC
No platelet clumping
Nodular sclerosing
HbSS
Histaminase and arylsulfatase

8. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Decrease EPO
Idiopathic thrombocytopenia (ITP)
SLE - CLL - alpha methyldopa
A antigen and B antibodies

9. Is G6PD intravascular or extravascular
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
All trans retinoic acid
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Intravascular

10. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
A antigen and B antibodies
Myelofibrosis (marow is crying being its fibrosed'
Ringed sideronblasts with iron laden mitochondria

11. Serum iron - transferrin - ferritin lab values for pregs - OCP use
B antigena and A antibodes
Nl - up - nl
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

12. What is the characteristic histologic finding in Hodgkin Lymphoma
Reed - sternberg cells
Hairy cell leukemia
Sideroblastic anemia
SLE - CLL - alpha methyldopa

13. What is the enzyme defect in Porphyria cutanea tarda
B cells
Uroporphyrinogen decarboxylase
CML
Deficiency in factor VIII

14. inc HbF on electrophoresis
Langerhans cells
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Multiple myeloma
Both alpha and beta thal

15. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Mantle cell lymphoma
Inc vasodiltion - inc perm - inc pain
Blasts > 5%

16. What is the Ddx for for a macrocytic anemia
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Uroporphyrin (tea colored urine)
Ferrocheletase and ALA dehydrogenase
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

17. What is the philadelphia chromosome
Von Willebrand's disease
T(9;22) bcr abl
Mantle cell - older males
Antigen - antibody

18. Deficiency in GpIb
Bernard soulier
Bimodal - men>women - except for nodular sclerosing type
CML to AML or All
Inc lymphoctes - and less RS cells

19. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Leukemia
Alpha thal - asian and african american
Her next Rh+ fetus
B6 therapy (pyrodixine)

20. What are the labs and tx for HS?
Hereditary spherocytosis - G6PD - sickle cell
Collagen - BM - activated platelets
Positive osmootic fragility test and splenectomy
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

21. Which drugs can cause macrocytic anemia?
5- FU - AZT - hydroxyurea
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
EBV

22. Sickle cell
Inc serum iron - normal TIBC - inc ferratin
Bernard soulier
Helminth infections major basic protein
Sickle cell anemia

23. Why can newborns with sickle cell be asymptomatic
Pos
Pyruvate kinase def - extravascular
Inc HbF and dec HbS
8-10 days

24. What is the treatment for sideroblastic anemia
Uroporphyrin (tea colored urine)
MGUS - monoclonal gammopathy of undetermined significance
B6 therapy (pyrodixine)
Porphyrias

25. What are some causes of cold agglutinin anemia
...
Sideroblastic anemia
Inc vasodiltion - inc perm - inc pain
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

26. From what cells are platelets derived from
CNS and testis
Megakaryocytes
Antigen - antibody
Mantle cell - older males

27. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Beta 4
Folate def
Inactivates it
CNS and testis

28. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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29. Basophilic nuclear remnants fonud in RBCs
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Ticlopidine/clopidogrel
ADP and Ca
Howell - Jolly bodies - hypo/asplenia

30. Where do B cells arise from - mature - and migrate to...
Helminth infections major basic protein
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Macrohemorrhage - hemarthroses - easy bruising

31. Where does All spread
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Nodular sclerosing
CNS and testis
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

32. What is the characteristic finding for MAHA on peripheral smear?
Round densly staining nucleus with a small amount of pale cytoplasm
Schistocytes - helmet cells
Porphyrias
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

33. What virus is associated with 50% of Hodgkin lymphoma
Lead - and EtOH
Fe def - thal - pb poison - sideroblastic anemia
T(12;21)
EBV

34. What are some classic examples of extravascular hemolysis
8-10 days
40 to 75
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Hereditary spherocytosis - G6PD - sickle cell

35. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Howell Jolly bodies
Folate def
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

36. S-100 and CD1a with birbeck granules
Beta chain underproduced - asymptomatic - heterozygote
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Langerhans cell histiocytosis
Plummer - vinson syndrome

37. What are the main associations with multiple myeloma?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Inappropriate absolute with inc RBCs and EPO
Down - up - down
B12 def

38. What is the therapy for CML (philly chrom)
Up - down - nl
Imatinib - anti bcr abl
Parvovirus
EBV

39. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
DIC
Adult T cell lymphoma - presents with cutaneous lesions
Sickle cell

40. What do you see in vit K def
Inc HbF and dec HbS
Pyruvate kinase def - extravascular
Eleveated PT - PTT
Langerhans cell histiocytosis

41. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Imatinib - anti bcr abl
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Lysine for glutamate at position 6

42. What are dendritic cells called in the skin?
Fibrinogen
Inappropriate absolute with inc RBCs and EPO
Langerhans cells
Glanzmann's throbmasthenia

43. Elderly - mature b cell tumor with filamentous - hairlike projections
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Hairy cell leukemia
TRAP (tartrate resistant acid phosphatase
Plasma cell

44. How does vWD cause elevated PTT?
VWF carries/protects factor VIII
ADP and Ca
Howell - Jolly bodies - hypo/asplenia
Burkitt lymphoma

45. What does 'Neutrophils Like Making Everything Better' stand for?
Dec plasma volume
...
No lytic bone lesions in WM
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

46. Bite cell
Plasma cell
G6PD
120 days
Porphobilinogen - delta ALA - uroporphyrin

47. What is factor V leidin?

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48. What is their role?
Sheets of lymphocytes interspersed with macs
Nodular sclerosing
Inducers of primary antibody response
No antigen - both antibodies

49. Wilm's tumor - RCC - HCC - hydronephrosis
Sideroblastic anemia
Ankryin - band 4.1 or spectrin
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Pyruvate kinase def - extravascular

50. Adults present with cutaneous patches/nodules - indolent CD4+
Signs and sx of aplastic anemia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Mycosis fundgoides/Sezary syndrome
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT