heme Flashcards
lead poisoning (MOA… leads to… )
- 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
most common primary cardiac tumor (name and histology, possible complication)
- atrial myxoma
- amorphous extracellular matrix
- associated with multiple syncopal episodes
CD4 marker
-helper T cells
CD19 marker
-B lymphocytes
CD28 marker
-helper T cells
CD3 marker
-helper T cells
CD16 marker
-natural killer cells
CD14 marker
-macrophages
G6PD (MOA and inciting drugs)
- 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
paroxysmal nocturnal hemoglobinuria (genetic defect, MOA)
- 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
blood smear of macrocytic anemia
- hypersegmented neutrophils
- can distinguish folate from B12 by neuro symptoms (only in B12) and increased MMA (only in B12)
SCID (enzyme deficiency… MOA… tx)
- 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
CML (genetics… MOA.. tx)
- t(9;22), philly chromosome, creates BCR-ABL fusion protein, constitutively active tyrosine kinase
- tx: TKI, imatinib (gleevec)
dacryocytes (tear drop cells on smear)
-indication of extramedullary hematopoeisis
von Willebrands (what is it, what does it do, lab values, inheritance)
- 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
protein C
-inactivation of factors V and VIII, deficiency leads to prothrombotic state
DIC (common causes… 2 fold mechanism… lab values… diagnostic test…)
- 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)
Burkitt’s Lymphoma (genetics, path, common symptom)
- 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
Folate deficiency (physiologic role of folate… what deficiency looks like, who gets it, time course)
- 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**