Pathology - hem/onc Flashcards
Acanthocyte
liver disease, abetalipoproteinemia
cholesterol dysregulation
Basophilic stippling
Lead poisoning
Degmacyte
G6PD deficiency
Elliptocyte
Hereditary elliptocytosis
Macro-ovalocyte
Megaloblastic anemia
Ringed sideroblast
excess iron in mitochondria
Schistocyte
DIC, TTP, HUS, HELLP
Sickle cell
Sickle cell
dehydration, deoxygenation, high altitude
Spherocyte
hereditary spherocytosis (incr. MCHC!)
Dacrocyte (teardrop cell)
Bone marrow infiltration (RBC squeezed out)
Target cell
HbC disease, Asplenia, Liver disease, Thalassemia
Heinz bodies
Oxidation of Hb-SH with subsequent phagocytic damage to RBC membrane (leads to bite cells)
G6PD deficiency
Howell-Jolly bodies
basophilic nuclear remnants found in RBCs
(normally removed by splenic macrophages)
sign of asplenia or functional asplenia (sickle-cell, polysplenia)
Microcytic, hypochromic anemias (5 total)
Iron deficiency Alpha-thalassemia Beta-thalessemia Lead poisoning Sideroblastic anemia
Lab tests in iron-deficiency anemia
decr. iron, incr. TIBC, decr. ferritin
look for conjunctival pallor and spool nails (koilonychia)
Gene deletions found in alpha-thalassemia
loss of alpha-globin gene
cis deletion = Asians
trans deletion = African
4 alleles deleted = incompatible with life
3 alleles deleted = very little alpha globin, HbH disease
Gene mutations found in beta-thalassemia
Point mutations in splice sites and promoter sequences
Minor: asymp. look for incr. HbA2
Major:
- symp after 6mos of age, req. blood transfusions
- marrow expansion –> bone deformities
- extramedullary hematopoiesis, incr. risk of parvob19 induced crisis
Lab/smear findings in lead poisoning
Lab: decr. heme synthesis, incr. protophorphyrin
Smear: rRNA aggregates –> basophilic stippling
Clinical findings of LEAD poisoning
Lead lines on x-ray: long bones and gingivae
Encephalopathy and RBC stippling
Abdominal colic and sideroblastic anemia
Drops - wrist and foot drop. Dimercaprol and EDTA
Causes of sideroblastic anemia
defect in heme synthesis
Genetic, acquired (myelodysplasia), reversible (alcohol, B6 deficiency, copper deficiency, isoniazid)
Use Prussian blue to stain iron-laden mitochondria
TX: B6/pyroxidine (provides co-factor)
Sideroblastic anemia labs
incr. iron, normal to decr. TIBC, incr. ferritin
4 causes of macrocytic anemia
Megaloblastic: folate deficiency, B12 deficiency, orotic aciduria
Nonmegaloblastic: unimpaired DAN synthesis (alcoholism, liver disease, hypothyroidism, reticulocytosis)
Mechanism of megaloblastic anemia
Impaired DNA synthesis –> maturation of nucleus of precursor cells in bone marrow is slower than the maturation of the cytoplasm
Leads to RBC macrocytosis and hypersegmented neutrophils
Folate vs. B12 deficiency
Folate = incr. homocysteine, normal MMA, no neuro sx
B12 = incr. homocysteine, incr. MMA, neuro sx “subacute combined degeneration”
Orotic aciduria vs. OTC deficiency
Orotic aciduria = defect in UMP synthase, no incr. NH4
OTC deficiency = incr. orotic acid and incr. NH4
Non-hemolytic normocytic normochromic anemia (2 total)
Anemia of chronic disease - incr. hepcidin, decr. iron release from macs (labs = decr. iron, decr. TIBC, incr. ferritin)
Aplastic anemia - failure or destruction of myeloid stem cells (radiation/drugs, viral agents, Fanconi anemia, idiopathic), fatty infiltration of marrow –> pancytopenia
Characteristics of intravascular hemolytic normocytic anemia
decr. haptoglobin, incr. LDH, schistocytes, incr. retics
think mechanical, PNG, MAHA
Characteristics of extravascular hemolytic normocytic anemia
Macs in spleen clear RBCs, so no Hgb in urine, incr. unconj bilirubin, jaundice
Ex. HS, PK deficiency, HbC defect, sickle cell anemia