heme Flashcards
(49 cards)
1
Q
lead poisoning (MOA… leads to… )
A
- inhibition of both aminolevulinic acid dehydratase (leads to increased precursor) and ferrochelatase (final step in heme synthesis)
- lead-induced porphyria leads to microcytic (hemoglobin poor) anemia
2
Q
most common primary cardiac tumor (name and histology, possible complication)
A
- atrial myxoma
- amorphous extracellular matrix
- associated with multiple syncopal episodes
3
Q
CD4 marker
A
-helper T cells
4
Q
CD19 marker
A
-B lymphocytes
5
Q
CD28 marker
A
-helper T cells
6
Q
CD3 marker
A
-helper T cells
7
Q
CD16 marker
A
-natural killer cells
8
Q
CD14 marker
A
-macrophages
9
Q
G6PD (MOA and inciting drugs)
A
- glucose-6-phosphatate dehydrogenase important enzyme in hexose monophosphate shunt
- reduces NADP+ to NADPH, which in turn reduces GSSG to glutathione–> glutathione protects RBC’s against oxidative stress
- deficiency results in hemolytic anemia
- common in AA and mediterranean descent
- primaquine, chloroquine, sulfa, Isoniozid
10
Q
paroxysmal nocturnal hemoglobinuria (genetic defect, MOA)
A
- defect in PIG-A gene which produces GPI anchors
- GPI anchors are necessary to attach complement inhibiting proteins, without which, complement chronically lyses RBCs
- occurs throughout the day, most concentrated urine in AM
11
Q
blood smear of macrocytic anemia
A
- hypersegmented neutrophils
- can distinguish folate from B12 by neuro symptoms (only in B12) and increased MMA (only in B12)
12
Q
SCID (enzyme deficiency… MOA… tx)
A
- adenosine deaminase deficiency
- inability to breakdown purines, buildup of deoxyadenosine triphosphate, which prevents synthesis of other nucleotides
- toxic to rapidly growing cells such as immune cells
- tx: bone marrow transplant
13
Q
CML (genetics… MOA.. tx)
A
- t(9;22), philly chromosome, creates BCR-ABL fusion protein, constitutively active tyrosine kinase
- tx: TKI, imatinib (gleevec)
14
Q
dacryocytes (tear drop cells on smear)
A
-indication of extramedullary hematopoeisis
15
Q
von Willebrands (what is it, what does it do, lab values, inheritance)
A
- 2 jobs: protect factor 8 and adhesion of platelets to collagen via glycoproteins
- increased bleeding time and partial thrombosplatin time
- NL prothrombin time and NL platelets
- autosomal dominant
16
Q
protein C
A
-inactivation of factors V and VIII, deficiency leads to prothrombotic state
17
Q
DIC (common causes… 2 fold mechanism… lab values… diagnostic test…)
A
- sepsis or trauma, but most commonly obstetric complications
- activation of BOTH coagulation cascade and fibrinolytic system, leading to…
- increased bleeding time, PT and PTT
- fibrin split products (D-dimer)
18
Q
Burkitt’s Lymphoma (genetics, path, common symptom)
A
- t(8;14) resulting in overexpression of c-myc
- starry sky, field of lymphocytes interrupted by occasional macrophages
- mass in jaw, and assoc. with EBV
19
Q
Folate deficiency (physiologic role of folate… what deficiency looks like, who gets it, time course)
A
- tetrahydrafolate is important in one carbon transfers, crucial for purine synthesis
- deficiency leads to megaloblastic microcytic anemia without neurological symptoms
- folate stores deplete quickly (time course of months rather than years in B12)
- *NL MMA**
20
Q
taut vs relaxed hemoglobin
A
- taut, tight, low oxygen, release oxygen, low affinity
- relaxed, lots of oxygen, pick it up and hold onto it, high affinity
21
Q
SLL/CLL vs diffuse large B-cell lymphoma (Path smear and pathogenicity)
A
- small, still mostly circular lymphocytes on smear
- Richter’s is a transformation from SLL/CLL to diffuse large cell (about 10% of SLL/CLL) and a smear shows a distinct line of transformation
- 4-5x larger cells and more disorganized morphology
22
Q
most common cause of hypercoaguable state (and 1 uncommon cause and 3 rare cases)
A
- factor V leiden
- prothrombin gene mutation
- protein s deficiency
- protein c deficiency
- antithrombin III deficiency
23
Q
CLL (path smear)
A
- clonal expansion of b-lymphocytes arrested in development somewhere between pre-b cell and mature b cell
- results in smudge cells as b cells are not fully mature and break apart during preparation
24
Q
HIT
A
- heparin induced thrombocytopenia
- antibodies form against heparin/platelet complex, activates platelets, they all quickly get used up forming lots of clots
- more likely in people who have been heparinized before
25
1 unit of raises hematocrit by....
-3%
26
thrombotic thrombocytopenic purpura (TTP)
- deficient vonWillebrand factor, leads to continual activation of platelets, destroys RBCs, leads to renal insufficiency
- this damage can lead to renal insufficiency and elevated creatinine
- NL PT and PTT, clotting factors are OK
27
vitamin K in clotting cascade
- vitamin K assits in carboxylating several clotting factors
| - warfarin interferes with this action
28
acute onset cardiac friction rub is an indication of....
-uremia, get them some dialysis pronto
29
contraindicated for warfarin
-pregnant women
30
hemophagocytic lymphohistiocytosis (HLH)
- constitutional symptoms
| - bone marrow aspirate reveals RBC's engulfed in macrophages
31
multiple myeloma (CRAB)
- hypoCalcemia
- Renal insufficiency
- Anemia
- Bone lesions
32
hairy cell leukemia (negative symptoms, smear, diagnostic test)
- neg for night sweats and fever
- 4x more common in men, fatigue, fullness, weight loss
- "hairy cell" on smear
- TRAP (tartrate resistant acid phosphatase) positive... trap the hairy animal
33
fanconi's anemia (what is it, why does it happen, what does it cause)
- most common congenital aplastic anemia and important cause of myeloid leukemia in kids
- defect in DNA repair
- bifid thumbs, renal dysgenesis, hypogonadism, microcephaly, high fetal hemoglobin
34
carboxylation of glutamic acid residues
-role of vitamin K in coag cascade
35
hemorrhagic disease of the newborn (what causes it, what prevents it)
- usually 2-7 days after birth, due to deficiency of vitamin K
- lacking intestinal flora that makes vitamin K
- every newborn in HOSPITAL gets vit K intramuscularly after birth
36
schilling test
-radiolabled B12–> in NL individuals, absorbed and excreted in urine, so we look for radiolabled B12 in urine, if not there, its not being absorbed (due to lack of intrinsic factor (produced by parietal cells) )
37
TTP-HUS (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome)
- a subtype of MAHA (microangiopathic hemolitic anemia) often idiopathic, sometimes caused by certain drugs bacterial toxins (shiga toxin from E. coli)
- trifecta of hemolytic anemia, thrombocytopenia and platelet aggregation
- tx is generally supportive, keeping an eye on fluids, maybe dialysis
38
hemophilia A and B (which cofactors are affected, genetics)
- hemophilia A: factor 8
- hemophilia B: factor 9
- both are X-linked recessive
39
PT and PTT measure which cofactors...
- PT: 7... also 10, 5, prothrombin and fibrinogen
| - PTT: 8, 9, 11, 12... also 10, 5, prothrombin and fibrinogen
40
hereditary spherocytosis (presentation (triad of symptoms), defect, and tx)
- anemia, splenomegaly, jaundice
- defect in RBC anchoring proteins: ankyrin, spectrin, band 3
- tx: splenectomy to cure anemia, sphereocytes persist
41
genetic abnormality in sickle cell disease (HbS)
-substitution of valine for glutamic acid at 6th position
42
henoch-schonlein (presentation, and serious complication)
- young boys, palpable purpuric lesions on buttocks and legs, joint pain, fever, malaise
- acute renal failure w/hematuria and proteinuria
43
post splenectomy, pts are at risk for... (4 pathogens with common trait..bonus, what will you see on smear?)
- encapsulated organisms: neisseria meningitides, strep pneumo, h. influenza, klebsiella pneumo
- given prophylactic vaccinations
- howell-jolly bodies, irregular RBCs, and target cells
44
bronze diabetes...
- jaundice and diabetes
| - hemochromatosis... leads to end organ damage
45
lymphocyte rich hodgkins lymphoma (smear, epidemiology, prognosis)
- few RS cells (binucleate with inclusion-like nucleoli
- usually young males
- decent prognosis
46
mixed cellularity hodgkins (smear, epidemiology, prognosis)
- many RS cells, many lymphocytes (mixed ratio)
- generally presents in older pts
- poor prognosis
47
plummer-vinson disease (triad)
- esophageal-cervical web
- iron deficiency anemia (microcytic hypochromic)
- glossitis
- MOA: dysphagia due to web and glossitis prevent proper intake, results in iron deficiency anemia
48
scurvy... (vitamin deficiency, symptoms and pathophys)
- deficient in vit C
- important in hydroxylating valine and leucine allowing for crosslinks which add structural integrity to collagen
- bleeding gums, poor wound healing etc
49
sickle cell trait... (common SE)
- much less severe than sickle cell disease, can see some more serious complications at altitude
- episodic hematuria and polyuria as renal tubules are damaged over time and body is unable to concentrate urine
