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Hemeonc

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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 percentage of WBCs are basophils - and What is found in their basophilic granules?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Off center nuclues - clock face chromatin
VIII
Glucose and heme - which inhibit ALA synthase

2. What vitamin is a cofactor for the first step of heme synthesis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Macrohemorrhage - hemarthroses - easy bruising
B6

3. What is the treatment for sideroblastic anemia
Von Willebrand's disease
Intravascular
B6 therapy (pyrodixine)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

4. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Nl PT - elevated PTT - intrinsic pathway defect
Leukemoid reaction
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Easy gas exchange

5. What is the tx for sickle cell
Hodgkin
Schistocytes and inc LDH
Up - down - up
Hydroxyurea - bone marrow transplant

6. What does hairy cell leukemia stain with
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Hairy cell leukemia
Sideroblastic anemia
TRAP (tartrate resistant acid phosphatase

7. After an injury - What does vWF bind to begin platelet plug formation?
Contiguous
T(12;21)
VWD
Exposed collagen upon endothelial damage

8. What is the main source of energy in RBCs
Nodular sclerosing
Burkitt lymphoma
90% anearobically from glucose to lactate - 10% from HMP shunt
EBV

9. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Plasma cell
Collagen - BM - activated platelets
Nl PT - elevated PTT - intrinsic pathway defect
Factor V Leiden

10. What does the large SA:volume ratio in RBCs help facilitate?
40 to 50
Dec
Easy gas exchange
MHC II and Fc receptors

11. What role does antithrombin play?
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Inc lymphoctes - and less RS cells
Incactivates II - VII - IX - X - XI - XII
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

12. What causes the physiologic chloride shift and What does the chloride shift do?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Petechiae
Varying sizes
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

13. What is the age group most commonly affected by multiple myeloma
Hb Barts - gamma4 in defect in all 4 alpha genes
AB - no antibodies
Sickle cell anemia
40 to 50

14. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Dec plasma volume
B12 def
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
T(9;22) bcr abl

15. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Beta chain underproduced - asymptomatic - heterozygote
A antigen and B antibodies
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

16. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
2 to 10
Protein C or S deficiency
Intravascular hemolysis

17. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Ferrocheletase and ALA dehydrogenase
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
AB - no antibodies
Polycythemia vera

18. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Plummer - vinson syndrome
G6PD
40 to 50

19. What activates the intrinsic pathway?
Hairy cell leukemia
B12 def
Sickle cell
Collagen - BM - activated platelets

20. What are the presenting symptoms of acute intermittent porphyria
Mantle cell lymphoma
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

21. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
No platelet clumping
Lead - and EtOH
Beta chain underproduced - asymptomatic - heterozygote
No lytic bone lesions in WM

22. What does decreasing heme do to ALA synthase activity?
Fibrinogen bind GpIIb/IIIa and links platelts
Down - down - up
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Inc

23. What is the age breakdown for hodgkins
Beta thal
TRAP (tartrate resistant acid phosphatase
Liver disease - abetalipoproteinemia - acntho = spiny
Bimodal - men>women - except for nodular sclerosing type

24. CD5+ - poor prognosis - t(11;14)
Bleeding - malnutrition/absorption - inc demand pregs
Leukemoid reaction
Mantle cell - older males
Bone marrow infiltration - myelofibrosis

25. What is the receptor for vWF
Burkitt lymphoma
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Up - down - up
Gp1b

26. Bite cell
Bone marrow infiltration - myelofibrosis
G6PD
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
B6

27. What is the characteristic histologic finding in Hodgkin Lymphoma
Ankryin - band 4.1 or spectrin
Reed - sternberg cells
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Up - down - up

28. Who has more severe disease - HbSS or HbSC
Factor V resistant to activated protein C's inhibition
Up - down - up
Up - down - nl
HbSS

29. What is the most common hodgkin lymphoma
Nodular sclerosing
Leukemoid reaction
No
Lymphoma

30. What causes hydrops fetalis
G6PD
Low in CML
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Hb Barts - gamma4 in defect in all 4 alpha genes

31. What is a blast crisis
G6PD
CML to AML or All
Intravascular
Multiple myeloma

32. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Hereditary elloptocytosis
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Beta thal

33. What does Vit K deficiency cause?
Varying sizes
Fibrinogen bind GpIIb/IIIa and links platelts
They bind vWF via GpIb
Dec synthesis of factors 1972 - protein C/S

34. Spherocyte
All
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Hereditary spherocytosis - autoimmune hemolysis

35. What are the labs and tx for HS?
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Positive osmootic fragility test and splenectomy
Leukemia
VWF and fibrinogen

36. Where are monocytes typically found - where do they go - and What do the differentiate into?
Eleveated PT - PTT
Paroxysmal nocturnal hemoglobinuria
No platelet clumping
Blod - tissue - MACS

37. What does bradykinin do?
Inc vasodiltion - inc perm - inc pain
Tissue
Cromolyn sodium
Blood

38. Where are 1/3 of platelets stored
Spleen
20 to 40
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Low in CML

39. Fava beans - sufla drugs - infectinos
Examples of oxidative stress
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
8-10 days

40. What is the life spance of a platelet?
Bone marrow infiltration - myelofibrosis
8-10 days
Hereditary spherocytosis - autoimmune hemolysis
Fc

41. What is the characteristic finding for MAHA on peripheral smear?
Schistocytes - helmet cells
Glanzmann's throbmasthenia
TRAP (tartrate resistant acid phosphatase
Gp1b

42. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Blistering cutaneous photosens - most common porphyria
Hereditary spherocytosis - autoimmune hemolysis
Easy gas exchange

43. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
B12 in fatty acid pathways leads to subacute combined degeneration
Ferrocheletase and ALA dehydrogenase
Tissue

44. t(11:22)
Fe def - thal - pb poison - sideroblastic anemia
T(12;21)
8-10 days
Ewing sarcoma

45. What does STOP Making New Thrombi stand for
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
MHC II and Fc receptors
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

46. Back pain - hemoglobinuria
Bite cells and Heinz bodies
No antigen - both antibodies
Common a few days after oxidative stress in a pt with G6PD
Ferrocheletase and ALA dehydrogenase

47. Can B cells function as APCs?
Down - up - down
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Yes - via MHC II
B6

48. What is the pathogenesis of ACD?
B12 def
G6PD
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

49. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Abciximab
All

50. What is the Ddx for a normocytic - normochromic anemia?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Myelofibrosis (marow is crying being its fibrosed'
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils