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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 portion of IgE can mast cells bind
Dendritic cells?
120 days
Fc
Orotic aciduria

2. universal donor
Her next Rh+ fetus
Beta chain underproduced - asymptomatic - heterozygote
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
No antigen - both antibodies

3. Why can newborns with sickle cell be asymptomatic
B symptoms - fever night sweats - weight loss
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Contiguous
Inc HbF and dec HbS

4. drug that inhibits ADP induced expression of GpIIb/IIIa
Def in factor IX
Ticlopidine/clopidogrel
Myelofibrosis (marow is crying being its fibrosed'
Sheets of lymphocytes interspersed with macs

5. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
Lung disease - congenital heart diseaes - and high altitude
Birbeck granules
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
All

6. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Diffuse Large b cell lymphoma
Sickle cell
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

7. t(11:22)
Incactivates II - VII - IX - X - XI - XII
Inactivates it
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Ewing sarcoma

8. What do auer rods stain with
Langerhans cells
Peroxidase
Uroporphyrinogen decarboxylase
Inappropriate absolute with inc RBCs and EPO

9. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
DIC - TTP/HUS - traumatic hemolysis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Rhogam - Rh antigen immunoglobulin

10. What does ADP do?
Myelofibrosis (marow is crying being its fibrosed'
Helps platelts adhere to endothelium
Varying sizes
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

11. What substances are released in mast cell degranulation
Inc UCB
Macrohemorrhage - hemarthroses - easy bruising
Histamine - heparin - and eosinohil chemotactic factors
Pos

12. What is the affected enzyme in lead poisoning
Dec
Extravascular hemolysis
Ferrocheletase and ALA dehydrogenase
Positive osmootic fragility test and splenectomy

13. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Inappropriate absolute with inc RBCs and EPO
Dec
TPA - cleavage of fibrin mesh
Protein C or S deficiency

14. What does ectopic EPO produce
Von Willebrand's disease
Low O2 in papilla; can also get microhematuria from medullary infarcts
Inappropriate absolute with inc RBCs and EPO
ADP and Ca

15. Serum iron - transferrin - ferritin lab values for pregs - OCP use
ATIII def
1 to 6
Nl - up - nl
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

16. Back pain - hemoglobinuria
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Common a few days after oxidative stress in a pt with G6PD
DIC
Hairy cell leukemia

17. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Helminth infections major basic protein
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
DDAVP (desmopressin) which releases stored vWF stored in endothelium

18. S-100 and CD1a with birbeck granules
Sheets of lymphocytes interspersed with macs
Langerhans cell histiocytosis
Ewing sarcoma
Both alpha and beta thal

19. What do labs show in ITP?
Hereditary elloptocytosis
Rhogam - Rh antigen immunoglobulin
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Sickle cell anemia

20. Elliptocyte
Hereditary elloptocytosis
B antigena and A antibodes
G6PD
Leukemia

21. What is the most common hodgkin lymphoma
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
MGUS - monoclonal gammopathy of undetermined significance
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Nodular sclerosing

22. What are the main associations with multiple myeloma?
Her next Rh+ fetus
Mycosis fundgoides/Sezary syndrome
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
B symptoms - fever night sweats - weight loss

23. Does CML have a JAK2 mut
Varying sizes
Inc lymphoctes - and less RS cells
No
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

24. What are dendritic cells called in the skin?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
No platelet clumping
Langerhans cells
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

25. What does STOP Making New Thrombi stand for
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Lacunar in nodular sclerosing variant
Birbeck granules

26. What does NAACP stand for - in regards to the causes of eosinophiia?
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Essential thrombocytosis
Her next Rh+ fetus

27. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Histaminase and arylsulfatase
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Anemia of chronic disease - Aplastic anemia - kidney disease
Megakaryocytes

28. What are some classic examples of extravascular hemolysis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Hereditary spherocytosis - G6PD - sickle cell
Tissue
Idiopathic thrombocytopenia (ITP)

29. hemolytic in a newborn - dec ATP and rigid RBCs
Pyruvate kinase def - extravascular
Lung disease - congenital heart diseaes - and high altitude
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Down - up - down

30. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Multiple myeloma
Direct coombs - agglutinate if RBCs are coated with Ig
Hereditary spherocytosis
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

31. adults - auer rods - inc circulating myeblasts on peripheral smear
Blood
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
AML
Exposed collagen upon endothelial damage

32. What is the therapy for the M3 variant?
Common a few days after oxidative stress in a pt with G6PD
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
All trans retinoic acid
VIII

33. What is the main source of energy in RBCs
B12/folate def
Aspirin
90% anearobically from glucose to lactate - 10% from HMP shunt
Fe def - thal - pb poison - sideroblastic anemia

34. Adults present with cutaneous patches/nodules - indolent CD4+
Inactivates it
Mycosis fundgoides/Sezary syndrome
Fibrinogen bind GpIIb/IIIa and links platelts
Dec synthesis of factors 1972 - protein C/S

35. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Hb Barts - gamma4 in defect in all 4 alpha genes
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Histaminase and arylsulfatase
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

36. What activates the intrinsic pathway?
Varying shapes
Collagen - BM - activated platelets
Porphobilinogen - delta ALA - uroporphyrin
AB - no antibodies

37. Who has more severe disease - HbSS or HbSC
20 to 40
Up - down - nl
HbSS
Leukemoid reaction

38. HTLV-1
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Adult T cell lymphoma - presents with cutaneous lesions
Von Willebrand's disease
Splicing sites and promotor sequences

39. What is the purpose of the fibrin mesh
Acts to stabilize platelet plug
Inc RBC - dec O2 sat - inc EPO
B6
Fibrinogen

40. What is the age breakdown for hodgkins
Bimodal - men>women - except for nodular sclerosing type
1 to 6
PGI2 - NO inc blood flow - dec platelet aggregation
TXA2 - dec blood flow - inc platelet aggregation

41. What is monoclonal expansion without symptoms associated with multiple myeloma?
All
Anemia of chronic disease - Aplastic anemia - kidney disease
Up - down - nl
MGUS - monoclonal gammopathy of undetermined significance

42. What is the receptor for vWF
Gp1b
40 to 50
Beta 4
Dec synthesis of factors 1972 - protein C/S

43. What do you see in vit K def
Eleveated PT - PTT
They bind vWF via GpIb
Africa = Jaw lesion - US = pelvis or abdomen
B symptoms - fever night sweats - weight loss

44. Bone pain plus anemia
A antigen and B antibodies
Multiple myeloma
Schistocytes and inc LDH
40 to 50

45. How are the nucleus and the cytoplasm characterized for lymphocytes
Inc large vWF multimers - inc platelet aggregation and thrombosis
No antigen - both antibodies
Inducers of primary antibody response
Round densly staining nucleus with a small amount of pale cytoplasm

46. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
Megakaryocytes
MAHA
Paroxysmal nocturnal hemoglobinuria

47. Conditions of defective heme synthesis leading to accumulation of heme precurors
Peroxidase
Signs and sx of aplastic anemia
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Porphyrias

48. What are the age ranges for the various leukemias
Reed - sternberg cells
Dendritic cells?
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Inducers of primary antibody response

49. What is the result of thrombocytopenia or platelet dysfunction?
Inc RBC - dec O2 sat - inc EPO
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Ferrocheletase and ALA dehydrogenase
Petechiae

50. Deficiency in GpIb
Bernard soulier
Inducers of primary antibody response
Lysosomes
Factor V resistant to activated protein C's inhibition