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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 is the treatment for sideroblastic anemia
Low O2 in papilla; can also get microhematuria from medullary infarcts
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
B6 therapy (pyrodixine)
DIC

2. hypocellular bone marrown tih fatty infiltration
Her next Rh+ fetus
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Aplastic anemia - pancytopenia

3. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Ringed sideronblasts with iron laden mitochondria
Extravascular hemolysis

4. What percentage of WBCs are monocytes?
Blasts > 5%
Schistocytes - helmet cells
Macrohemorrhage - hemarthroses - easy bruising
2 to 10

5. t(11:22)
They bind vWF via GpIb
No platelet clumping
Ewing sarcoma
A antigen and B antibodies

6. What is the Ddx for a normocytic - normochromic anemia?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Anti Rh
Lymphoma
Examples of oxidative stress

7. Which immunoglobulin is involved in warm agglutination?
1 to 6
IgG - Warm is GREAT
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
...

8. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
Bone marrow infiltration - myelofibrosis
T(12;21)
Macrohemorrhage - hemarthroses - easy bruising

9. Bone pain plus anemia
Multiple myeloma
Inc
Incactivates II - VII - IX - X - XI - XII
MAHA

10. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Nl - up - nl
All
Hereditary spherocytosis - autoimmune hemolysis
Hereditary spherocytosis

11. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Birbeck granules
Porphyrias
Signs and sx of aplastic anemia
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 -

12. philadelphia chromosome - blood looks like marrow
Pos
CML
Idiopathic thrombocytopenia (ITP)
Ankryin - band 4.1 or spectrin

13. bcl -2 t(14;18) adults
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Follicular lymphoma - indolent course

14. Basophilica stippling
Leukemoid reaction
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Africa = Jaw lesion - US = pelvis or abdomen

15. What causes the physiologic chloride shift and What does the chloride shift do?
40 to 75
Sheets of lymphocytes interspersed with macs
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Paraprotein spike - monoclonal protein

16. Where are basophils found?
Extravascular
Blood
Eleveated PT - PTT
Ferrocheletase and ALA dehydrogenase

17. What does bradykinin do?
Nl - up - nl
Hodgkin
Inc vasodiltion - inc perm - inc pain
Myelofibrosis (marow is crying being its fibrosed'

18. What is the age breakdown for hodgkins
Inc HbF and dec HbS
Extravascular
Bimodal - men>women - except for nodular sclerosing type
Essential thrombocytosis

19. What vitamin is a cofactor for the first step of heme synthesis
B6
MHC II and Fc receptors
Factor V Leiden
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

20. What is factor V leidin?

21. What virus can cause an aplastic crisis in pts with HS?
B symptoms - fever night sweats - weight loss
8-10 days
Parvovirus
Heinz bodies - seen in alpha thal and G6PD

22. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Parvovirus
Protein C or S deficiency
VIII
Sideroblastic anemia

23. What substance is Fe added to to yield heme
Petechiae
Birbeck granules
Intravascular hemolysis
Protoporphyrin

24. Which substrates begin the heme synthesis pathway
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inc
Glycine and succinyl - coa
Von Willebrand's disease

25. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

26. Blood type B
B antigena and A antibodes
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Hereditary spherocytosis
Anti Rh

27. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Antigen - antibody
Inducers of primary antibody response
Birbeck granules

28. Where to T cells arise and mature - and migrate to...
Reed - sternberg cells
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Ringed sideronblasts with iron laden mitochondria
Decrease EPO

29. Which infections can cause MAHA?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Malaria - Babesia
PGI2 - NO inc blood flow - dec platelet aggregation
Tissue

30. What is the therapy for the M3 variant?
DIC
VWF and fibrinogen
All trans retinoic acid
Mycosis fundgoides/Sezary syndrome

31. What is monoclonal expansion without symptoms associated with multiple myeloma?
Idiopathic thrombocytopenia (ITP)
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
MGUS - monoclonal gammopathy of undetermined significance
Petechiae

32. What does the large SA:volume ratio in RBCs help facilitate?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Varying sizes
Beta chain underproduced - asymptomatic - heterozygote
Easy gas exchange

33. Basophilic nuclear remnants fonud in RBCs
Leukemia
Mantle cell lymphoma
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Howell - Jolly bodies - hypo/asplenia

34. What does increasing heme do to ALA synthase activity
Dec
VWF carries/protects factor VIII
Hereditary spherocytosis - G6PD - sickle cell
Burkitt lymphoma

35. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Essential thrombocytosis
Her next Rh+ fetus
Sideroblastic anemia
Helminth infections major basic protein

36. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Paroxysmal nocturnal hemoglobinuria
EBV
Porphyrias
Complications of sickle cell anemia

37. What does STOP Making New Thrombi stand for
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
VWD
HIV or immunosupression

38. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
M3 AML (acute promyelocytic leukemia)
Protein C or S deficiency
Intravascular

39. Is HS extravascular or intravascular?
Extravascular
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
No platelet clumping
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

40. What substances are released in mast cell degranulation
Histamine - heparin - and eosinohil chemotactic factors
Direct coombs - agglutinate if RBCs are coated with Ig
Dendritic cells?
Leukemia

41. What is hemophiliia A
Deficiency in factor VIII
Follicular lymphoma - indolent course
Burkitt lymphoma
Up - down - nl

42. What is the effect of ACE on bradykinin
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Lung disease - congenital heart diseaes - and high altitude
Bleeding - malnutrition/absorption - inc demand pregs
Inactivates it

43. What does ectopic EPO produce
Essential thrombocytosis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inappropriate absolute with inc RBCs and EPO
B6

44. What are the labs and tx for HS?
All trans retinoic acid
EBV
Blood
Positive osmootic fragility test and splenectomy

45. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
All trans retinoic acid
Von Willebrand's disease
Fe def - thal - pb poison - sideroblastic anemia

46. What are the likely exposures of kids and adults for lead poisoning
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Kids= exposure to lead paint - adults = battery - ammunition factory
Schistocytes and inc LDH
Dec synthesis of factors 1972 - protein C/S

47. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Schistocytes and inc LDH
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Down - up - down
A antigen and B antibodies

48. What are dendritic cells called in the skin?
1 to 6
Langerhans cells
Down - down - up
Porphobilinogen deaminase aka uroporphyrinogen I synthase

49. Where do B cells arise from - mature - and migrate to...
Ewing sarcoma
Anemia of chronic disease - Aplastic anemia - kidney disease
Def in factor IX
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

50. What is the difference of presentation of Burkitt in Africa vs the United States
Fc
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Hairy cell leukemia
Africa = Jaw lesion - US = pelvis or abdomen