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Test your basic knowledge |
Hemeonc
Start Test
Study First
Subject
:
health-sciences
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. deficiency in ADAMTS13 leading to dec degradation of vWF
Bimodal - men>women - except for nodular sclerosing type
All
Thrombotic thrombocytopenic purpura
Nl - up - nl
2. How does the therapy for M3 vairant work?
No antigen - both antibodies
Induces differentiation of myeloblasts
Pos
2 to 10
3. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
They bind vWF via GpIb
2 to 10
4. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Incactivates II - VII - IX - X - XI - XII
Low in CML
No platelet clumping
Alpha thal - asian and african american
5. Upregulated growth of leukocytes in bone marro
Megakaryocytes
Intravascular hemolysis
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Leukemia
6. 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 HbA2 on electrophoresis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
CLL (SLL without the peripheral lymphocytosis
7. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
MGUS - monoclonal gammopathy of undetermined significance
Mantle cell - older males
No antigen - both antibodies
Factor V Leiden
8. What is the presenting scenario for TTP?
Off center nuclues - clock face chromatin
Acts to stabilize platelet plug
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
SLE - CLL - alpha methyldopa
9. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Peroxidase
Africa = Jaw lesion - US = pelvis or abdomen
Hodgkin
No lytic bone lesions in WM
10. What do you see on peripheral smear with sideroblastic anemia
Hereditary spherocytosis - autoimmune hemolysis
Inc serum iron - normal TIBC - inc ferratin
Ringed sideronblasts with iron laden mitochondria
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
11. What is the result of thrombocytopenia or platelet dysfunction?
T(9;22) bcr abl
Petechiae
Lacunar in nodular sclerosing variant
DIC
12. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
G6PD
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Inc lymphoctes - and less RS cells
13. What virus is associated with 50% of Hodgkin lymphoma
Beta chain underproduced - asymptomatic - heterozygote
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 -
Kids= exposure to lead paint - adults = battery - ammunition factory
EBV
14. What are the neuro sx of B12 def?
Sickle cell anemia
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Direct coombs - agglutinate if RBCs are coated with Ig
Cromolyn sodium
15. What happens in betal thal minor?
Inc RBC - dec O2 sat - inc EPO
MAHA
Hereditary spherocytosis
Beta chain underproduced - asymptomatic - heterozygote
16. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Up - down - nl
Off center nuclues - clock face chromatin
Uroporphyrin (tea colored urine)
17. Teardrop cell
Complications of sickle cell anemia
Histamine - heparin - and eosinohil chemotactic factors
Bone marrow infiltration - myelofibrosis
Africa = Jaw lesion - US = pelvis or abdomen
18. What are the main associations with multiple myeloma?
Blasts > 5%
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
EBV
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
19. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Tissue
Megakaryocytes
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Folate def
20. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Beta thal
Sickle cell anemia
G6PD
21. What are some classic examples of extravascular hemolysis
Antigen - antibody
Langerhans cells
No antigen - both antibodies
Hereditary spherocytosis - G6PD - sickle cell
22. what proteins can be defective in HS?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Ankryin - band 4.1 or spectrin
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Plasma cell
23. What do eosinophils defend against and What do they use to do it?
Intrinsic - all factors except - VII - XIII
Follicular lymphoma - indolent course
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Helminth infections major basic protein
24. What is the pathogenesis of TTP?
TXA2 - dec blood flow - inc platelet aggregation
Liver disease - abetalipoproteinemia - acntho = spiny
Dec
Inc large vWF multimers - inc platelet aggregation and thrombosis
25. What is the therapy for CML (philly chrom)
Lead - and EtOH
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Gp1b
Imatinib - anti bcr abl
26. What portion of IgE can mast cells bind
Protoporphyrin
Beta thal
Fc
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
27. What does increasing heme do to ALA synthase activity
Aspirin
Schistocytes - helmet cells
Dec
DIC
28. t(11:22)
Inc
TXA2 - dec blood flow - inc platelet aggregation
Ewing sarcoma
Inc lymphoctes - and less RS cells
29. What does decreasing heme do to ALA synthase activity?
Abciximab
Positive osmootic fragility test and splenectomy
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Inc
30. Which infections can cause MAHA?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Malaria - Babesia
Macrohemorrhage - hemarthroses - easy bruising
GpIIb/IIIa
31. philadelphia chromosome - blood looks like marrow
HIV or immunosupression
Def in factor IX
CLL (SLL without the peripheral lymphocytosis
CML
32. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Sideroblastic anemia
Acts to stabilize platelet plug
ATIII def
Indirect coombs - agglutinate if serum anti RBC surface Ig
33. What are dendritic cells called in the skin?
SLE - CLL - alpha methyldopa
Essential thrombocytosis
Def in factor IX
Langerhans cells
34. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Lacunar in nodular sclerosing variant
Idiopathic thrombocytopenia (ITP)
No platelet clumping
Antigen - antibody
35. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Idiopathic thrombocytopenia (ITP)
Bone marrow - thymus - blood (80% of circulating lymphos are T)
ATIII def
MAHA
36. Where are 1/3 of platelets stored
20 to 40
...
Spleen
CML
37. Deficiency in GpIIb/IIIa
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38. Sickle cell
Plummer - vinson syndrome
Mantle cell lymphoma
Sickle cell anemia
Reed - sternberg cells
39. What does STOP Making New Thrombi stand for
Extrinsic - I - II - V - VII and X
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Complications of sickle cell anemia
Hereditary spherocytosis - autoimmune hemolysis
40. What is the age breakdown for hodgkins
Bimodal - men>women - except for nodular sclerosing type
MHC II and Fc receptors
Complications of sickle cell anemia
ATIII def
41. What are some causes of cold agglutinin anemia
Dec plasma volume
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
ATIII def
Schistocytes and inc LDH
42. Which substrates begin the heme synthesis pathway
Blod - tissue - MACS
Epoxide reductase - warfarin inhibits
Glycine and succinyl - coa
Hereditary spherocytosis - autoimmune hemolysis
43. What is the clinical picture of hemophilia A or B
90% anearobically from glucose to lactate - 10% from HMP shunt
Tissue factor converst the VII and VIIa
Macrohemorrhage - hemarthroses - easy bruising
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
44. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
B12 def
Degrades fibrin mesh and converts C3 to C3a
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inc vasodiltion - inc perm - inc pain
45. What is the effect of ACE on bradykinin
Ringed sideronblasts with iron laden mitochondria
Inactivates it
Bone marrow infiltration - myelofibrosis
Incactivates II - VII - IX - X - XI - XII
46. What percentage of WBCs are eosinophils?
1 to 6
Cromolyn sodium
Plasma cell
ATIII def
47. Why can newborns with sickle cell be asymptomatic
IgG - Warm is GREAT
Inc HbF and dec HbS
TPA - cleavage of fibrin mesh
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
48. Serum iron - transferrin - ferritin lab values for hemochromatosis
Up - down - up
Examples of oxidative stress
VWD
Low in CML
49. What is the ddx for aplastic anemia
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50. When do you see MAHA?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Cromolyn sodium
Mantle cell - older males
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis