17. Heme Flashcards
What coagulation factor is deficient in hemophilia A?
8
What coagulation factor is deficient in hemophilia B?
9
What is the clinical consequence of a deficiency in either protein C or protein S?
hypercoagulable
What is the treatment for overdose of heparin?
protamine sulfate
What is the treatment for heparin-induced thrombocytopenia?
- stop heparin
- start different anticoagulant
What allows RBCs to change shape as they pass through vessels?
spectrin
What name is given to immature erythrocytes in circulation?
reticulocytes
What pathologic form of RBC would you see in each of the following diseases?
- lead poisoning
basophilic stippling
What pathologic form of RBC would you see in each of the following diseases?
- G6PD deficiency
Heinz bodies –> bite cells
What pathologic form of RBC would you see in each of the following diseases?
- DIC
schistocytes
What pathologic form of RBC would you see in each of the following diseases?
- abetalipoproteinemia
acanthocyte (spur cell)
What pathologic form of RBC would you see in each of the following diseases?
- asplenia
Howell-Jolly bodies
Target cells
Where does fetal erythropoiesis take place?
Yolk sac –> liver –> spleen –> BM
In which adult bones does erythropoiesis take place?
axial skeleton: vertebrae, ribs, pelvis
What are some of the diff. causes of polycythemia? (4)
- ectopic EPO (pheo, RCC, HCC, hemangioblastoma)
- chronic hypoxia
- polycythemia vera
- trisomy 21
What are the hematologic and non-hematologic findings in a patient with lead poisoning?
hematologic: microcytic anemia, basophilic stippling
non-heme: encephalopathy, memory loss, HA, foot/wrist drop, lead lines on gingivae, abdominal colic, renal failure
What test can be used to diagnose beta-thalassemia minor?
Hb electrophoresis (will see increased HbA2)
What should you r/o in a man over 50 with new-onset iron deficiency anemia?
colon cancer
A patient is diagnosed with a macrocytic, megaloblastic anemia. What is the danger of giving folate alone?
uncorrected B12 def –> peripheral neuropathy
What is the cause of anemia?
- microcytic anemia + swallowing difficulty + glossitis
Plummer Vinson syndrome
What is the cause of anemia?
- microcytic anemia, >3.5% HbA2
beta-thal minor
What is the cause of anemia?
- megaloblastic anemia not correctable by B12 or folate
orotic aciduria
What is the cause of anemia?
- megaloblastic anemia along with peripheral neuropathy
b12 deficiency
What is the cause of anemia?
- microcytic anemia + basophilic stippling
lead poisoning
What is the cause of anemia?
- microcytic anemia reversible with B6
sideroblastic anemia (note: B6 is cofactor for delta-ALA synthase)
What is the cause of anemia?
- HIV-positive patient with macrocytic anemia
zidovudine (HIV drug)
What is the cause of anemia?
- normocytic anemia and elevated creatinine
chronic kidney disease
A child anemic since birth has now been cured with splenectomy. What is the disease?
hereditary spherocytosis
What findings are associated with hereditary spherocytosis?
anemia, jaundice (b/c extravascular), pigmented gall stones, splenomegaly
- (+) osmotic fragility test
- (-) Coombs
What is the difference b/w hemoglobin S defect and hemoglobin C?
HbS (sickle cell) glu –> valine
HbC: glu –> lysine
What is the difference b/w warm agglutinin and cold agglutinin
warm: IgG
cold: IgM
What are schistocytes?
fragmented RBCs by mechanical destruction
In what hematologic disorder would you find each of the following abnormal tests/findings?
- Heinz bodies
G6PD
In what hematologic disorder would you find each of the following abnormal tests/findings?
- osmotic fragility test
hereditary spherocytosis
What is the life span of a platelet (which is also the max life of platelets after a transfusion)?
8-10 days
What molecule is expressed on the surface of a platelet after it becomes activated?
GP IIb/IIIa
NSAIDs inhibit the production of which substance important in platelet aggregation?
Thromboxane A2
What is the MoA of each of the following drugs?
- Streptokinase
thrombolytic (converts plasminogen –> plasmin) –> cleaves fibrin
What is the MoA of each of the following drugs?
- aspirin
irreversibly inhibits COX1 and 2
What is the MoA of each of the following drugs?
- clopidogrel
blocks ADP receptors
What is the MoA of each of the following drugs?
- abciximab
binds GP IIb/IIIa on platelets
What is the MoA of each of the following drugs?
- tirofiban
binds GP IIb/IIIa on platelets
What is the MoA of each of the following drugs?
- ticlopidine
blocks ADP receptors
What is the MoA of each of the following drugs?
- enoxaparin
LMWH (inhibit factor 10a)
What is the MoA of each of the following drugs?
- eptifibatide
GP IIb/IIIa inhibitor
What is the cause of ITP?
antibodies to glycoprotein IIb/IIIa on platelets (which then gets removed from circulation)
What is the defect in Bernard-Soulier disease?
deficient GP1b –> defect in plt-to-vWF adhesion
After a normal spontaneous vaginal delivery, the new mom bleeds profusely from her vagina and later from her gums. What abnormal lab values would you suspect?
- decreased PC
- increased BT, PT, PTT
- increased d-dimer, decreased fibrinogen
Compare the age distribution of those affected by Hodgkin lymphoma to those affected by non-Hodgkin lymphoma
HL: bimodal (peak @ age 20, 65)
NHL: variable
What is the most common type of non-Hodgkin lymphoma in adults? In children?
adults: diffuse large B cell lymphoma
children: lymphoblastic lymphoma
What form of lymphoma matches each of the following statements?
- most common lymphoma in US
diffuse large B-cell lymphoma
What form of lymphoma matches each of the following statements?
- reed-sternberg cells
hodgkin lymphoma
What form of lymphoma matches each of the following statements?
- particularly associated with EBV
Burkitt lymphoma, hodgkin lymphoma
What form of lymphoma matches each of the following statements?
- associated with long-term celiac disease
intestinal T-cell lymphoma
What form of lymphoma matches each of the following statements?
- lymphoma equivalent of CLL
small lymphocytic lymphoma
What form of lymphoma matches each of the following statements?
- “starry sky” pattern due to phagocytosis of apoptotic tumor cells
burkitt
What form of lymphoma matches each of the following statements?
- associated with sjogren syndrome, hashimoto thyroiditis, and H. pylori
marginal cell MALToma
A patient presents with anemia, hypercalcemia, and bone pain undergoes a bone marrow biopsy, which reveals plasma cells. What is the diagnosis and what may be found on urinalysis?
Multiple myeloma
- no proteinuria on standard UA
- need UPEP for detection of Bence Jones proteins
What form of leukemia matches each of the following?
- most common leukemia in children
ALL
What form of leukemia matches each of the following?
- most common leukemia in adults in US
CLL
What form of leukemia matches each of the following?
- characteristic Auer rods
AML (M3 type)
What form of leukemia matches each of the following?
- myelodysplastic syndromes have a tendency to progress to ___
AML
What form of leukemia matches each of the following?
- myeloproliferative disorders may progress to ___
AML
What form of leukemia matches each of the following?
- more than 20% blasts in marrow
acute leukemia
What form of leukemia matches each of the following?
- leukemia with more mature cells and
chronic leukemia
What form of leukemia matches each of the following?
- TdT (+) acute leukemia
ALL
What form of leukemia matches each of the following?
- commonly presents with bone pain
ALL
What form of leukemia matches each of the following?
- numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase
CML
What form of leukemia matches each of the following?
- always positive for the philadelphia chromosome (t9;22)
CML
What form of leukemia matches each of the following?
- acute leukemia positive for peroxidase
AML
What form of leukemia matches each of the following?
- solid sheets of lymphoblasts in marrow
ALL
What form of leukemia matches each of the following?
- always associated with the bcr-abl gene
CML
