Test your basic knowledge |

Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
If you are not ready to take this test, you can study here.
Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.

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 pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
CLL (SLL without the peripheral lymphocytosis
Splicing sites and promotor sequences
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

2. What virus is associated with 50% of Hodgkin lymphoma
EBV
They bind vWF via GpIb
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
B antigena and A antibodes

3. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Hodgkin
TPA - cleavage of fibrin mesh
Aplastic anemia - pancytopenia
Lymphoma

4. What symptoms are associated with Hodgkin lymphoma
Incactivates II - VII - IX - X - XI - XII
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Down - down - up
B symptoms - fever night sweats - weight loss

5. Deficiency in GpIb
Splicing sites and promotor sequences
Bernard soulier
Howell Jolly bodies
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

6. What is the enzyme defect in Porphyria cutanea tarda
Dec synthesis of factors 1972 - protein C/S
MGUS - monoclonal gammopathy of undetermined significance
Inc UCB
Uroporphyrinogen decarboxylase

7. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Dec
They bind vWF via GpIb
Polycythemia vera

8. What is the Ddx for nonhemolytic normocytic anemia
Kids= exposure to lead paint - adults = battery - ammunition factory
T(9;22) bcr abl
Anemia of chronic disease - Aplastic anemia - kidney disease
Bone marrow infiltration - myelofibrosis

9. What is the age group most commonly affected by multiple myeloma
Beta thal
40 to 50
Glanzmann's throbmasthenia
B12/folate def

10. What vitamin is a cofactor for the first step of heme synthesis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Indirect coombs - agglutinate if serum anti RBC surface Ig
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
B6

11. What portion of IgE can mast cells bind
T(9;22) bcr abl
Inc
Fc
CML to AML or All

12. Which pathway and factors are tested in the PTT test
20 to 40
Intrinsic - all factors except - VII - XIII
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
DIC - TTP/HUS - traumatic hemolysis

13. What is the treatment for sideroblastic anemia
Intravascular hemolysis
Mantle cell lymphoma
No antigen - both antibodies
B6 therapy (pyrodixine)

14. Where does All spread
CNS and testis
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Examples of oxidative stress
Langerhans cell histiocytosis

15. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Fibrinogen
CLL (SLL without the peripheral lymphocytosis
Cromolyn sodium
Ringed sideronblasts with iron laden mitochondria

16. What is their role?
Plasma cell
Down - down - up
Inducers of primary antibody response
CML

17. What is the tx for vWD
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Paraprotein spike - monoclonal protein
No lytic bone lesions in WM

18. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
Anti Rh
ATIII def
Porphobilinogen deaminase aka uroporphyrinogen I synthase

19. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Contiguous
Macrohemorrhage - hemarthroses - easy bruising
VIII

20. What is the therapy for CML (philly chrom)
Common a few days after oxidative stress in a pt with G6PD
Imatinib - anti bcr abl
Dec plasma volume
SLE - CLL - alpha methyldopa

21. Blood type A
Mantle cell - older males
SLE - CLL - alpha methyldopa
Birbeck granules
A antigen and B antibodies

22. What are the four levels of alpha thal?
Liver disease - abetalipoproteinemia - acntho = spiny
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Sickle cell 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 -

23. What do the iron studies show in sideroblastic anemia
Factor V resistant to activated protein C's inhibition
Hereditary spherocytosis
Inc serum iron - normal TIBC - inc ferratin
Sideroblastic anemia

24. What is the tx for sickle cell
Hydroxyurea - bone marrow transplant
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Fc

25. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Birbeck granules
All trans retinoic acid
Langerhans cell histiocytosis

26. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Inc UCB
Burkitt lymphoma
Fe def - thal - pb poison - sideroblastic anemia
Heinz bodies - seen in alpha thal and G6PD

27. HTLV-1
Extravascular
Adult T cell lymphoma - presents with cutaneous lesions
1 to 6
CML

28. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
CD15 and CD30 pos
Sideroblastic anemia
Myelofibrosis (marow is crying being its fibrosed'
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

29. What percentage of WBCs are monocytes?
Myelofibrosis (marow is crying being its fibrosed'
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
2 to 10
Hairy cell leukemia

30. t(11:22)
Lysine for glutamate at position 6
Fibrinogen bind GpIIb/IIIa and links platelts
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Ewing sarcoma

31. Which cell is neoplastic in multiple myeloma
Lymphoma
Plasma cell
ADP and Ca
Sideroblastic anemia

32. What is a metabolic disorder tht can cause macrocytic anemia?
Uroporphyrin (tea colored urine)
Orotic aciduria
MAHA
T(12;21)

33. What is the defect in beta thal?
Sickle cell anemia
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Splicing sites and promotor sequences

34. What do the platelets bind? What is the step called
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Megakaryocytes
They bind vWF via GpIb

35. Who are the professional APCs?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Inc UCB
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Dendritic cells?

36. What is the pathogenesis of ACD?
Lead - and EtOH
Leukemia
Incactivates II - VII - IX - X - XI - XII
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

37. What are the anti aggregation factors?
TRAP (tartrate resistant acid phosphatase
PGI2 - NO inc blood flow - dec platelet aggregation
Induces differentiation of myeloblasts
Essential thrombocytosis

38. What does Vit K deficiency cause?
B12 in fatty acid pathways leads to subacute combined degeneration
Heinz bodies - seen in alpha thal and G6PD
Von Willebrand's disease
Dec synthesis of factors 1972 - protein C/S

39. What is the accumulated substance in lead poisoning
Produce antibody - lots of RER and golgi
Beta chain underproduced - asymptomatic - heterozygote
All trans retinoic acid
Protoporphyrin (blood)

40. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Lysosomes
Sheets of lymphocytes interspersed with macs
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
B antigena and A antibodes

41. What does the large SA:volume ratio in RBCs help facilitate?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Easy gas exchange
Positive osmootic fragility test and splenectomy
Protoporphyrin (blood)

42. What does CRAB stand for in multiple myeloma
CD15 and CD30 pos
Her next Rh+ fetus
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Leukemia

43. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
Petechiae
Megakaryocytes
Helminth infections major basic protein

44. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Protein C or S deficiency
Hereditary elloptocytosis
B6
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

45. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Nodular sclerosing
Idiopathic thrombocytopenia (ITP)
Alpha thal - asian and african american
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

46. What happens in betal thal minor?
ATIII def
Beta 4
Lymphoma
Beta chain underproduced - asymptomatic - heterozygote

47. What is the receptor for vWF
Gp1b
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Exposed collagen upon endothelial damage
Factor V Leiden

48. How do platelet disorders present?
VIII
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Hb Barts - gamma4 in defect in all 4 alpha genes
Lung disease - congenital heart diseaes - and high altitude

49. What is the life span of a normal RBC
Inc HbF and dec HbS
120 days
Blod - tissue - MACS
Sideroblastic anemia

50. mother's antibodies attack fetal RBCs
B antigena and A antibodes
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
ATIII def
Erythroblastosis fetalis - Rh or other blood antigen incompatibility