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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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1. Fava beans - sufla drugs - infectinos
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Examples of oxidative stress
Sheets of lymphocytes interspersed with macs
DIC - TTP/HUS - traumatic hemolysis

2. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
40 to 50
B cells
Schistocytes - helmet cells

3. tennis rackets on EM
Porphyrias
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Birbeck granules
Alpha thal - asian and african american

4. What are the pro aggregation factors?
TXA2 - dec blood flow - inc platelet aggregation
Rhogam - Rh antigen immunoglobulin
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Schistocytes - helmet cells

5. What does the large SA:volume ratio in RBCs help facilitate?
Nl PT - elevated PTT - intrinsic pathway defect
90% anearobically from glucose to lactate - 10% from HMP shunt
Easy gas exchange
IFN gama

6. adults - auer rods - inc circulating myeblasts on peripheral smear
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
G6PD
Parvovirus
AML

7. S-100 and CD1a with birbeck granules
120 days
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Langerhans cell histiocytosis
1 to 6

8. What is HbH
Beta 4
Paroxysmal nocturnal hemoglobinuria
TPA - cleavage of fibrin mesh
Induces differentiation of myeloblasts

9. Does CML have a JAK2 mut
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Inc HbF and dec HbS
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
No

10. deficiency in ADAMTS13 leading to dec degradation of vWF
Thrombotic thrombocytopenic purpura
CML
...
No antigen - both antibodies

11. Who has more severe disease - HbSS or HbSC
HbSS
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Decrease EPO
Extrinsic - I - II - V - VII and X

12. When do you see MAHA?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
PGI2 - NO inc blood flow - dec platelet aggregation
Inc RBC - dec O2 sat - inc EPO
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

13. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Extravascular hemolysis

14. What is the age group most commonly affected by multiple myeloma
Dec plasma volume
G6PD
40 to 50
Megakaryocytes

15. What is the aggregation phase of ppf?
Fibrinogen bind GpIIb/IIIa and links platelts
Deficiency in factor VIII
Down - up - down
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

16. normal RBCs added to patient's serum
Off center nuclues - clock face chromatin
Indirect coombs - agglutinate if serum anti RBC surface Ig
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Ferrocheletase and ALA dehydrogenase

17. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Liver disease - abetalipoproteinemia - acntho = spiny
Paroxysmal nocturnal hemoglobinuria
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
DIC

18. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
B12 in fatty acid pathways leads to subacute combined degeneration
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Paroxysmal nocturnal hemoglobinuria

19. What is the effect of ACE on bradykinin
Ringed sideronblasts with iron laden mitochondria
Inactivates it
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Mycosis fundgoides/Sezary syndrome

20. What converts plasminogen to plasm and What does plasmin do?
Cromolyn sodium
Off center nuclues - clock face chromatin
TPA - cleavage of fibrin mesh
G6PD

21. What does plasmin do?
Peroxidase
Inc UCB
Degrades fibrin mesh and converts C3 to C3a
Liver disease - abetalipoproteinemia - acntho = spiny

22. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Inc HbF and dec HbS
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Paroxysmal nocturnal hemoglobinuria
Blasts > 5%

23. When is the peak incidence for nonHod lymphoma
Lead - and EtOH
20 to 40
Inc RBC - dec O2 sat - inc EPO
Protoporphyrin (blood)

24. What is the life span of a normal RBC
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Low O2 in papilla; can also get microhematuria from medullary infarcts
TRAP (tartrate resistant acid phosphatase
120 days

25. What is the tx for aplastic anemia
Inc serum iron - normal TIBC - inc ferratin
Tissue factor converst the VII and VIIa
Inc lymphoctes - and less RS cells
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

26. What condition can result from treating AML M3 from the release of the Auer rods
Mantle cell lymphoma
Collagen - BM - activated platelets
Porphobilinogen - delta ALA - uroporphyrin
DIC

27. Which substrates begin the heme synthesis pathway
ADP and Ca
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Both alpha and beta thal
Glycine and succinyl - coa

28. hypocellular bone marrown tih fatty infiltration
Aplastic anemia - pancytopenia
Yes - via MHC II
Mantle cell lymphoma
Blood

29. Basophilic nuclear remnants fonud in RBCs
Varying shapes
Petechiae
Bernard soulier
Howell - Jolly bodies - hypo/asplenia

30. What is the activation stage of platelet plug formation?
G6PD
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

31. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Blistering cutaneous photosens - most common porphyria
Megakaryocytes
Hereditary spherocytosis
CML

32. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Reed - sternberg cells
Lysine for glutamate at position 6
Polycythemia vera

33. Serum iron - transferrin - ferritin lab values for iron def anemia
Down - up - down
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Blistering cutaneous photosens - most common porphyria
Hodgkin

34. CD5+ - poor prognosis - t(11;14)
Mantle cell - older males
AB - no antibodies
Bimodal - men>women - except for nodular sclerosing type
Paraprotein spike - monoclonal protein

35. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Plummer - vinson syndrome
Def in factor IX
Epoxide reductase - warfarin inhibits
Aplastic anemia - pancytopenia

36. From what cells are platelets derived from
Bite cells and Heinz bodies
Glucose and heme - which inhibit ALA synthase
Leukemia
Megakaryocytes

37. Eosinophils are highly phagocytic For what kind of complex?
Degrades fibrin mesh and converts C3 to C3a
Howell - Jolly bodies - hypo/asplenia
B symptoms - fever night sweats - weight loss
Antigen - antibody

38. What do you see on peripheral smear with sideroblastic anemia
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Ringed sideronblasts with iron laden mitochondria
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

39. What does STOP Making New Thrombi stand for
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Collagen - BM - activated platelets
All trans retinoic acid

40. What is their role?
Inducers of primary antibody response
Africa = Jaw lesion - US = pelvis or abdomen
Glanzmann's throbmasthenia
Fe def - thal - pb poison - sideroblastic anemia

41. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Protoporphyrin
Ringed sideronblasts with iron laden mitochondria
Beta thal

42. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Birbeck granules
Rhogam - Rh antigen immunoglobulin
Lead - and EtOH
Histaminase and arylsulfatase

43. bcl -2 t(14;18) adults
Incactivates II - VII - IX - X - XI - XII
Multiple myeloma
90% anearobically from glucose to lactate - 10% from HMP shunt
Follicular lymphoma - indolent course

44. What is the most common nonhodgkin lymphoma
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Diffuse Large b cell lymphoma
No lytic bone lesions in WM
Uroporphyrinogen decarboxylase

45. Back pain - hemoglobinuria
Common a few days after oxidative stress in a pt with G6PD
Multiple myeloma
B antigena and A antibodes
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

46. In hemophilia A or B What do you see in the coag tests
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Nl PT - elevated PTT - intrinsic pathway defect
TXA2 - dec blood flow - inc platelet aggregation
Malaria - Babesia

47. What is the most common hodgkin lymphoma
Hereditary elloptocytosis
Howell Jolly bodies
Nodular sclerosing
B antigena and A antibodes

48. Where are monocytes typically found - where do they go - and What do the differentiate into?
Blod - tissue - MACS
40 to 50
MAHA
Uroporphyrinogen decarboxylase

49. What is the treatment for acute intermittent porphyria
Inactivates it
Lymphoma
Cromolyn sodium
Glucose and heme - which inhibit ALA synthase

50. What does the blood smear show in glanzmann's?
No platelet clumping
Liver disease - abetalipoproteinemia - acntho = spiny
Bone marrow infiltration - myelofibrosis
Inc large vWF multimers - inc platelet aggregation and thrombosis