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Hemeonc

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Answer 50 questions in 15 minutes.
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1. What symptoms are associated with Hodgkin lymphoma
B symptoms - fever night sweats - weight loss
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
B antigena and A antibodes
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

2. What is the defect in beta thal?
VWF and fibrinogen
Idiopathic thrombocytopenia (ITP)
Splicing sites and promotor sequences
...

3. What are the likely exposures of kids and adults for lead poisoning
Plummer - vinson syndrome
Leukemia
Kids= exposure to lead paint - adults = battery - ammunition factory
Inc HbF and dec HbS

4. What is the treatment for acute intermittent porphyria
Idiopathic thrombocytopenia (ITP)
Glucose and heme - which inhibit ALA synthase
Direct coombs - agglutinate if RBCs are coated with Ig
Paraprotein spike - monoclonal protein

5. Who has more severe disease - HbSS or HbSC
HbSS
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Multiple myeloma
Plummer - vinson syndrome

6. Serum iron - transferrin - ferritin lab values for hemochromatosis
Blasts > 5%
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sickle cell anemia
Up - down - up

7. What percentage of WBCs are basophils - and What is found in their basophilic granules?
B12/folate def
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Paraprotein spike - monoclonal protein
Def in factor IX

8. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Complications of sickle cell anemia
Round densly staining nucleus with a small amount of pale cytoplasm
5- FU - AZT - hydroxyurea
Direct coombs - agglutinate if RBCs are coated with Ig

9. What are the age ranges for the various leukemias
MHC II and Fc receptors
B6 therapy (pyrodixine)
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Factor V Leiden

10. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Signs and sx of aplastic anemia
Down - down - up
Paroxysmal nocturnal hemoglobinuria
Both alpha and beta thal

11. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Fibrinogen
Parvovirus
Intravascular hemolysis
Polycythemia vera

12. Blood type B
B antigena and A antibodes
Macrohemorrhage - hemarthroses - easy bruising
Glucose and heme - which inhibit ALA synthase
Prothrombin gene mutation

13. What is the tx for sickle cell
Reed - sternberg cells
Inc
Idiopathic thrombocytopenia (ITP)
Hydroxyurea - bone marrow transplant

14. Which cell is neoplastic in multiple myeloma
Splicing sites and promotor sequences
Bernard soulier
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Plasma cell

15. What is the receptor for vWF
40 to 75
Gp1b
8-10 days
A antigen and B antibodies

16. What are the presenting symptoms of porphyria cutanea tarda
ADP and Ca
Blistering cutaneous photosens - most common porphyria
Inc HbF and dec HbS
CLL (SLL without the peripheral lymphocytosis

17. What are some causes of cold agglutinin anemia
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Parvovirus
CML to AML or All

18. What is contained within the azurophilic granules of PMNs
CLL (SLL without the peripheral lymphocytosis
MAHA
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Tissue

19. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Aplastic anemia - pancytopenia
8-10 days
Low in CML
Lacunar in nodular sclerosing variant

20. Where do you see hypersegmented polys?
Tissue
120 days
Ferrocheletase and ALA dehydrogenase
B12/folate def

21. What is the general pathology of a macrocytic anemia?
DIC
Cromolyn sodium
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Liver disease - abetalipoproteinemia - acntho = spiny

22. What are the four levels of alpha thal?
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
MGUS - monoclonal gammopathy of undetermined significance
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Sickle cell

23. anisocytosis
Inc RBC - dec O2 sat - inc EPO
Multiple myeloma
Varying sizes
Porphobilinogen - delta ALA - uroporphyrin

24. What is factor V leidin?

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25. What does ADP do?
2 to 10
M3 AML (acute promyelocytic leukemia)
Inc RBC - dec O2 sat - inc EPO
Helps platelts adhere to endothelium

26. What are dendritic cells called in the skin?
Langerhans cells
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Megakaryocytes
Up - down - nl

27. Ddx for microcytic anemia
B6 therapy (pyrodixine)
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Fe def - thal - pb poison - sideroblastic anemia
Parvovirus

28. What is relative polycythemia
Ferrocheletase and ALA dehydrogenase
MGUS - monoclonal gammopathy of undetermined significance
Dec plasma volume
Examples of oxidative stress

29. Adults present with cutaneous patches/nodules - indolent CD4+
Idiopathic thrombocytopenia (ITP)
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
B12/folate def
Mycosis fundgoides/Sezary syndrome

30. Schisotcyte - helmet cell
Blood
2 to 10
DIC - TTP/HUS - traumatic hemolysis
Schistocytes - helmet cells

31. How does the therapy for M3 vairant work?
Easy gas exchange
DIC
Induces differentiation of myeloblasts
Lysosomes

32. What do you see in peripheral smear in a pt with G6PD?
Bite cells and Heinz bodies
Leukemia
MAHA
IFN gama

33. mother's antibodies attack fetal RBCs
40 to 75
Howell - Jolly bodies - hypo/asplenia
VIII
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

34. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Her next Rh+ fetus
Easy gas exchange
Schistocytes and inc LDH
Inc

35. What activates the fibrinolytic pathway?
Inc serum iron - normal TIBC - inc ferratin
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Intravascular hemolysis

36. What are the labs and tx for HS?
GpIIb/IIIa
120 days
Positive osmootic fragility test and splenectomy
Bleeding - malnutrition/absorption - inc demand pregs

37. What is makes a leukemia acute
Blasts > 5%
Imatinib - anti bcr abl
1 to 6
Sideroblastic anemia

38. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Direct coombs - agglutinate if RBCs are coated with Ig
Up - down - nl

39. t(11:22)
No
Ewing sarcoma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Induces differentiation of myeloblasts

40. What does the blood smear show in glanzmann's?
Abciximab
Degrades fibrin mesh and converts C3 to C3a
No platelet clumping
B6

41. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Sickle cell
Plasma cell
Bone marrow - thymus - blood (80% of circulating lymphos are T)

42. t(8;14) c - myc gene
Orotic aciduria
Pos
Burkitt lymphoma
No lytic bone lesions in WM

43. Is G6PD intravascular or extravascular
T(9;22) bcr abl
Abciximab
ADP and Ca
Intravascular

44. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Lysine for glutamate at position 6
VWF carries/protects factor VIII
Birbeck granules
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

45. Is HS extravascular or intravascular?
Eleveated PT - PTT
Fc
Helminth infections major basic protein
Extravascular

46. What condition can result from treating AML M3 from the release of the Auer rods
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Kids= exposure to lead paint - adults = battery - ammunition factory
DIC
CLL (SLL without the peripheral lymphocytosis

47. What is the characteristic lab finding on electrophoresis
Heinz bodies - seen in alpha thal and G6PD
Sideroblastic anemia
Paraprotein spike - monoclonal protein
Orotic aciduria

48. Why does B12 def cause neuro sx?
Extrinsic - I - II - V - VII and X
B12 in fatty acid pathways leads to subacute combined degeneration
ADP and Ca
Idiopathic thrombocytopenia (ITP)

49. Teardrop cell
Hb Barts - gamma4 in defect in all 4 alpha genes
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Bone marrow infiltration - myelofibrosis

50. What does 'Neutrophils Like Making Everything Better' stand for?
No
Porphyrias
Porphobilinogen deaminase aka uroporphyrinogen I synthase
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

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Hemeonc

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