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Hemeonc

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1. HTLV-1
Adult T cell lymphoma - presents with cutaneous lesions
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Bite cells and Heinz bodies
CNS and testis

2. What substance accumulates in porphyria cutanea
Uroporphyrin (tea colored urine)
Blistering cutaneous photosens - most common porphyria
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Fibrinogen

3. What the alpha granules contain in platelets?
Her next Rh+ fetus
Aspirin
Tissue
VWF and fibrinogen

4. When do you see MAHA?
AML
HbSS
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
G6PD

5. What are the neuro sx of B12 def?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inc large vWF multimers - inc platelet aggregation and thrombosis
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Easy gas exchange

6. What is the pathogenesis of aplastic anemia with kidney disease
Factor V Leiden
Fibrinogen
Decrease EPO
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

7. How are the nucleus and the cytoplasm characterized for lymphocytes
Anemia of chronic disease - Aplastic anemia - kidney disease
Round densly staining nucleus with a small amount of pale cytoplasm
Up - down - nl
TPA - cleavage of fibrin mesh

8. Fibrotic obliteration of bone marow with teardrop cells

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9. What is the characteristic histologic finding in Hodgkin Lymphoma
Parvovirus
Leukemia
Uroporphyrin (tea colored urine)
Reed - sternberg cells

10. What do they express on their surface?
EBV
MHC II and Fc receptors
Anti Rh
Nl PT - elevated PTT - intrinsic pathway defect

11. What do platelets release necessary for coagulation cascaed?
Inc UCB
ADP and Ca
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
MHC II and Fc receptors

12. What is the philadelphia chromosome
90% anearobically from glucose to lactate - 10% from HMP shunt
T(9;22) bcr abl
1 to 6
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

13. What is the life span of a normal RBC
Burkitt lymphoma
120 days
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Extravascular hemolysis

14. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Inc RBC - dec O2 sat - inc EPO
Factor V Leiden
Aplastic anemia - pancytopenia
Acts to stabilize platelet plug

15. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Pos
Ringed sideronblasts with iron laden mitochondria
Nl - up - nl
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

16. 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)
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Birbeck granules
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

17. Where are basophils found?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Blood
Plummer - vinson syndrome

18. Adults present with cutaneous patches/nodules - indolent CD4+
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 -
Fe def - thal - pb poison - sideroblastic anemia
Mycosis fundgoides/Sezary syndrome
IgG - Warm is GREAT

19. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Up - down - nl
Paroxysmal nocturnal hemoglobinuria
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Inc serum iron - normal TIBC - inc ferratin

20. Who has more severe disease - HbSS or HbSC
Ringed sideronblasts with iron laden mitochondria
Paraprotein spike - monoclonal protein
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
HbSS

21. Conditions of defective heme synthesis leading to accumulation of heme precurors
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Glucose and heme - which inhibit ALA synthase
Porphyrias
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

22. inc LDH - jaundice
Myelofibrosis (marow is crying being its fibrosed'
Extravascular hemolysis
Lysosomes
Her next Rh+ fetus

23. Why does B12 def cause neuro sx?
Hereditary spherocytosis - autoimmune hemolysis
Plummer - vinson syndrome
Inc RBC - dec O2 sat - inc EPO
B12 in fatty acid pathways leads to subacute combined degeneration

24. Where do you see hypersegmented polys?
B12/folate def
Induces differentiation of myeloblasts
GpIIb/IIIa
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

25. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
B antigena and A antibodes
Hereditary spherocytosis - autoimmune hemolysis
Multiple myeloma

26. What is the main source of energy in RBCs
90% anearobically from glucose to lactate - 10% from HMP shunt
EBV
Beta thal
Essential thrombocytosis

27. deficiency in ADAMTS13 leading to dec degradation of vWF
Thrombotic thrombocytopenic purpura
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Ringed sideronblasts with iron laden mitochondria
They bind vWF via GpIb

28. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
...
All
Fe def - thal - pb poison - sideroblastic anemia

29. What do the platelets bind? What is the step called
Complications of sickle cell anemia
PGI2 - NO inc blood flow - dec platelet aggregation
CML
They bind vWF via GpIb

30. 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
Nl PT - elevated PTT - intrinsic pathway defect
Up - down - up
Von Willebrand's disease
Sickle cell

31. What is the treatment for lead poising?
No platelet clumping
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
DIC

32. What is the effect of ACE on bradykinin
Plasma cell
Sideroblastic anemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inactivates it

33. What do the labs show for TTP?
Complications of sickle cell anemia
1 to 6
Schistocytes and inc LDH
Megakaryocytes

34. What does STOP Making New Thrombi stand for
Hereditary spherocytosis
Beta 4
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

35. What does Vit K deficiency cause?
CD15 and CD30 pos
Dec
Down - down - up
Dec synthesis of factors 1972 - protein C/S

36. Blood type B
EBV
B antigena and A antibodes
DIC - TTP/HUS - traumatic hemolysis
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

37. What are the extrinsic hemolytic normocytic anemias?
Histamine - heparin - and eosinohil chemotactic factors
TRAP (tartrate resistant acid phosphatase
...
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

38. What reveresible things can a sideroblastic anemia
Pos
Lead - and EtOH
No antigen - both antibodies
All trans retinoic acid

39. What is monoclonal expansion without symptoms associated with multiple myeloma?
Leukemia
MGUS - monoclonal gammopathy of undetermined significance
Positive osmootic fragility test and splenectomy
ATIII def

40. In hemophilia A or B What do you see in the coag tests
Ticlopidine/clopidogrel
Folate def
Peroxidase
Nl PT - elevated PTT - intrinsic pathway defect

41. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Macrohemorrhage - hemarthroses - easy bruising
Sideroblastic anemia
Africa = Jaw lesion - US = pelvis or abdomen
Porphyrias

42. drug that inhibits ADP induced expression of GpIIb/IIIa
Aspirin
Liver disease - abetalipoproteinemia - acntho = spiny
Ticlopidine/clopidogrel
MHC II and Fc receptors

43. What virus is associated with Burkitt lymphoma
EBV
All
PGI2 - NO inc blood flow - dec platelet aggregation
Helps platelts adhere to endothelium

44. Which pathway and factorrs are tested by the PT coag test
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Extrinsic - I - II - V - VII and X
Round densly staining nucleus with a small amount of pale cytoplasm
Factor V resistant to activated protein C's inhibition

45. What is the difference of presentation of Burkitt in Africa vs the United States
Africa = Jaw lesion - US = pelvis or abdomen
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Mycosis fundgoides/Sezary syndrome
Epoxide reductase - warfarin inhibits

46. discrete tumor masses arising from lymph nodes
Macrohemorrhage - hemarthroses - easy bruising
Helminth infections major basic protein
Lymphoma
T(9;22) bcr abl

47. What are the four levels of alpha thal?
Porphobilinogen - delta ALA - uroporphyrin
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Glucose and heme - which inhibit ALA synthase
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

48. What is the receptor for vWF
Prothrombin gene mutation
Tissue
Extrinsic - I - II - V - VII and X
Gp1b

49. After an injury - What does vWF bind to begin platelet plug formation?
40 to 75
IFN gama
MGUS - monoclonal gammopathy of undetermined significance
Exposed collagen upon endothelial damage

50. anisocytosis
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Deficiency in factor VIII
Liver disease - abetalipoproteinemia - acntho = spiny
Varying sizes

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