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Hemeonc

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1. What does STOP Making New Thrombi stand for
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Deficiency in factor VIII
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
M3 AML (acute promyelocytic leukemia)

2. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Degrades fibrin mesh and converts C3 to C3a
Complications of sickle cell anemia
Easy gas exchange

3. What is the treatment for acute intermittent porphyria
Langerhans cells
Glucose and heme - which inhibit ALA synthase
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
IgG - Warm is GREAT

4. Who are the professional APCs?
Extrinsic - I - II - V - VII and X
Dendritic cells?
Sideroblastic anemia
Inducers of primary antibody response

5. What condition can result from treating AML M3 from the release of the Auer rods
Diffuse Large b cell lymphoma
T(12;21)
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
DIC

6. What is makes a leukemia acute
Protoporphyrin (blood)
Beta 4
Blasts > 5%
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

7. Why can newborns with sickle cell be asymptomatic
Inc HbF and dec HbS
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Low O2 in papilla; can also get microhematuria from medullary infarcts
Inc HbA2 on electrophoresis

8. What causes hydrops fetalis
Common a few days after oxidative stress in a pt with G6PD
Hereditary elloptocytosis
CNS and testis
Hb Barts - gamma4 in defect in all 4 alpha genes

9. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Anti Rh
Hb Barts - gamma4 in defect in all 4 alpha genes
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Sideroblastic anemia

10. What is the accumulated substance in lead poisoning
Beta chain underproduced - asymptomatic - heterozygote
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
40 to 50
Protoporphyrin (blood)

11. What does decreasing heme do to ALA synthase activity?
Inc
Protein S - cleaves and invactivates Va and VIIIa
40 to 75
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

12. How is beta thal minor dx
Inc HbA2 on electrophoresis
MGUS - monoclonal gammopathy of undetermined significance
Reed - sternberg cells
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

13. What is the pathogenesis of TTP?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inc large vWF multimers - inc platelet aggregation and thrombosis
Inc vasodiltion - inc perm - inc pain
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -

14. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Dec synthesis of factors 1972 - protein C/S
VWD
Paroxysmal nocturnal hemoglobinuria
Complications of sickle cell anemia

15. What is the main source of energy in RBCs
40 to 75
90% anearobically from glucose to lactate - 10% from HMP shunt
M3 AML (acute promyelocytic leukemia)
Tissue

16. Which infections can cause MAHA?
Malaria - Babesia
Imatinib - anti bcr abl
Fibrinogen bind GpIIb/IIIa and links platelts
Bleeding - malnutrition/absorption - inc demand pregs

17. What is the life spance of a platelet?
8-10 days
Langerhans cell histiocytosis
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Tissue

18. What activates the fibrinolytic pathway?
Macrohemorrhage - hemarthroses - easy bruising
Varying shapes
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

19. What is relative polycythemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Dec plasma volume
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
CML to AML or All

20. After an injury - What does vWF bind to begin platelet plug formation?
Uroporphyrinogen decarboxylase
Round densly staining nucleus with a small amount of pale cytoplasm
Exposed collagen upon endothelial damage
Extrinsic - I - II - V - VII and X

21. What is appropriate absolute polycythemia
Howell Jolly bodies
Inducers of primary antibody response
Idiopathic thrombocytopenia (ITP)
Inc RBC - dec O2 sat - inc EPO

22. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
Inappropriate absolute with inc RBCs and EPO
Up - down - up
Langerhans cell histiocytosis

23. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
VIII
Common a few days after oxidative stress in a pt with G6PD

24. Where do you see hypersegmented polys?
Adult T cell lymphoma - presents with cutaneous lesions
B12/folate def
DIC
CNS and testis

25. What is the pathogenesis of ACD?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Cromolyn sodium
Direct coombs - agglutinate if RBCs are coated with Ig

26. Acanthocyte (spur cell)
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Blood
Liver disease - abetalipoproteinemia - acntho = spiny
Cromolyn sodium

27. What is the ddx for aplastic anemia

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28. What CD molecules are on RS cells
CD15 and CD30 pos
CNS and testis
Intrinsic - all factors except - VII - XIII
Contiguous

29. 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
Sickle cell
Inc lymphoctes - and less RS cells
B cells
Nl PT - elevated PTT - intrinsic pathway defect

30. What do you see on peripheral smear with sideroblastic anemia
Sideroblastic anemia
Extrinsic - I - II - V - VII and X
Ringed sideronblasts with iron laden mitochondria
B12/folate def

31. Does CML have a JAK2 mut
Prothrombin gene mutation
No
Nodular sclerosing
Fibrinogen bind GpIIb/IIIa and links platelts

32. What is the characteristic spread of Hodgkin Lymphoma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Howell Jolly bodies
Contiguous
Up - down - nl

33. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Eleveated PT - PTT
Aplastic anemia - pancytopenia
...

34. What is the age group most commonly affected by multiple myeloma
40 to 50
Macrohemorrhage - hemarthroses - easy bruising
Peroxidase
EBV

35. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Africa = Jaw lesion - US = pelvis or abdomen
Blood
Inc HbF and dec HbS

36. Which pathway and factorrs are tested by the PT coag test
Protoporphyrin (blood)
Extrinsic - I - II - V - VII and X
Varying sizes
Tissue

37. What do platelets interact with to form a hemostatic plug
Induces differentiation of myeloblasts
Fibrinogen
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Hereditary spherocytosis - autoimmune hemolysis

38. What happens in betal thal minor?
Beta chain underproduced - asymptomatic - heterozygote
Degrades fibrin mesh and converts C3 to C3a
They bind vWF via GpIb
Valine for glutamate

39. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Epoxide reductase - warfarin inhibits
Low in CML
B antigena and A antibodes
SLE - CLL - alpha methyldopa

40. What percentage if WBCs are polys?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Ewing sarcoma
40 to 75
Bleeding - malnutrition/absorption - inc demand pregs

41. What does bradykinin do?
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
A antigen and B antibodies
Hb Barts - gamma4 in defect in all 4 alpha genes
Inc vasodiltion - inc perm - inc pain

42. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Essential thrombocytosis
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
5- FU - AZT - hydroxyurea
Polycythemia vera

43. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Down - down - up
Tissue
Histaminase and arylsulfatase
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

44. Schisotcyte - helmet cell
IFN gama
Inc lymphoctes - and less RS cells
ATIII def
DIC - TTP/HUS - traumatic hemolysis

45. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
Follicular lymphoma - indolent course
Protein C or S deficiency
B symptoms - fever night sweats - weight loss

46. t(11:22)
40 to 75
Dec plasma volume
Ewing sarcoma
All trans retinoic acid

47. How does vWD cause elevated PTT?
VWF carries/protects factor VIII
Von Willebrand's disease
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

48. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Anti Rh
Inc HbF and dec HbS
Epoxide reductase - warfarin inhibits
Her next Rh+ fetus

49. What is factor V leidin?

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50. t(11;14)
Mantle cell lymphoma
ADP and Ca
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Bimodal - men>women - except for nodular sclerosing type

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Hemeonc

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