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My Pathology > RBC Disorders > Flashcards

RBC Disorders Flashcards

1
Q

What is the significance of the corrected reticulocyte count?

A

Indicates effective erythropoiesis (good marrow response to anemia) if ≥3%

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2
Q

Extramedullary hematopoiesis

A
  • Erythropoiesis outside of bone marrow, often in liver or spleen
  • Due to intrinsic bone marrow disease or accelerated erythropoiesis (ex. with severe hemolysis)
  • Hepatosplenomegaly, “hair-on-end” sign on skull XR
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3
Q

Anemia

A
  • Decreased Hb, Hct, or RBC
  • Normal SaO2 and PaO2
  • Pulmonary valve murmur, pallor, high-output cardiac failure (from decreased viscosity)
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4
Q

What does increased RDW (RBC distribution width) indicate?

A

Iron-deficiency anemia

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5
Q

What do iron studies look like in anemia of chronic disease?

A
  • Increased serum ferritin

* Decreased serum iron, TIBC (transferrin), iron saturation

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6
Q

What are the 4 chains in each HbA, HbF, HbH, and Hb Bart?

A
  • HbA: 2 alpha, 2 beta
  • HbF: 2 alpha, 2 gamma
  • HbH: 4 beta
  • Hb Bart: 4 gamma
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7
Q

Causes of iron deficiency anemia

A

Inadequate iron intake, menorrhagia, GI blood loss, pregnancy/lactation, premature infants, celiac sprue, hemolysis

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8
Q

Clinical findings in iron deficiency anemia

A
  • Microcytic
  • Causative: esophageal web, achlorhydria (low stomach acid)
  • Associated: glossitis, angular cheilosis, koilonychia (spoon nails)
  • Conjunctival pallor, palmar creases, craving (pica) for rice
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9
Q

Lab findings in iron deficiency anemia

A
  • Decreased MCV, serum iron, serum ferritin, iron saturation
  • Increased TIBC, RDW, FEP
  • Thrombocytosis
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10
Q

Anemia of chronic disease

A
  • Chronic inflammation, alcoholism, malignancy
  • Normal or decreased MCV
  • Increased serum ferritin, FEP
  • Decreased serum iron, TIBC, iron saturation
  • Treat with EPO or hepcidin antagonist
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11
Q

Alpha thalassemia

A
  • Black population have 1 deletion on each chromosome
  • Southeast Asian population has 2 deletions on one chromosome
  • 3 deletions = HbH, causing severe hemolytic anemia
  • 4 deletions = Hb Bart, incompatible with life
  • Decreased MCV, Hb, Hct
  • Increased RBC
  • Normal RDW, serum ferritin, FDP
  • May show target or teardrop cells
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12
Q

Beta thalassemia minor

A
  • Mild microcytic anemia
  • Decreased MCV, Hb, Hct
  • Increased RBC
  • Normal RDW, serum ferritin, FDP
  • Target and teardrop cells present
  • Hb electrophoresis: increased HbA2 (2 alpha/2 delta) and HbF
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13
Q

Beta thalassemia major (Cooley anemia)

A
  • Severe hemolytic anemia; RBCs with alpha-chain inclusions removed by splenic macrophages
  • Jaundice, increased UCB
  • Extramedullary hematopoiesis: hepatosplenomegaly, “hair-on-end” skull XR
  • Increased RDW
  • Reticulocytes, teardrop cells, Howell-Jolly bodies (nuclear remnants), nucleated RBCs
  • Hb electrophoresis: no HbA, only HbA2 and HbF
  • Blood transfusion (danger of iron overload, requires chelation) or bone marrow transplant
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14
Q

Sideroblastic anemia

A
  • Alcoholism, B6 deficiency, lead poisoning, XLR inherited
  • Deficiency in heme synthesis of developing RBCs; iron accumulates and RBCs die
  • Children: abdominal colic, constipation, encephalopathy, growth retardation (lead in epiphyses)
  • Peripheral neuropathy, kidney damage, lead lines in guns
  • Increased serum iron, iron sat, ferritin; decreased MCV, TIBC
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15
Q

Megaloblastic anemia

A
  • Macrocytic
  • Folic acid or B12 deficiency (increased homocysteine levels)
  • Impaired DNA synthesis leads to enlarged, nucleated RBCs that are eventually destroyed
  • Oval macrocytes
  • Pernicious anemia may cause B12 deficiency; Abs vs parietal cells, type 2 hypersensitivity, achlorhydria; yellow skin, associated w/ other autoimmune diseases (test: Abs vs intrinsic factor binding)
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16
Q

Nonmegaloblastic macrocytosis

A
  • Macrocytes round, not oval
  • Normal WBCs, platelets
  • Liver disease from alcohol: no anemia
  • Alcohol alone: mild anemia, reversible
17
Q

Aplastic anemia

A
  • 15-25 yrs old or >60 yrs old
  • Immune destruction of myeloid stem cells; sometimes TERT mutation
  • Fever, bleeding, fatigue
  • Pancytopenia, reticulocytopenia, hypocellular marrow
  • Pure RBC aplasia possible from leukemia drugs, parvovirus
18
Q

Chronic renal failure

A
  • ACD and decreased EPO
  • Burr cells (RBC with undulating membrane)
  • Prolonged bleeding time
19
Q

Extrinsic hemolytic anemia

A
  • RBCs destroyed by macrophages in spleen

* Increased UCB, LDH

20
Q

Intrinsic hemolytic anemia

A
  • RBCs destroyed in vessel

* Increased plasma and urine Hb, hemosiderinuria

21
Q

Hereditary spherocytosis

A
  • Autosomal dominant
  • Spectrin (membrane protein) defect yields spherocyte RBCs, which are destroyed in spleen
  • Jaundice, gallstones, splenomegaly
  • Normocytic anemia, increased RDW
  • Treat with splenectomy
22
Q

Hereditary elliptocytosis

A
  • Autosomal dominant
  • Membrane protein defect
  • May cause mild hemolytic anemia, splenomegaly
  • Splenectomy for symptomatic pts
23
Q

Paroxysmal nocturnal hemoglobinuria

A
  • Acquired stem cell mutation
  • Complement-mediated lysis of RBCs, neutrophils, and platelets at night (aided by resp acidosis)
  • Possible iron deficiency leading to microcytic anemia, risk of vessel thrombosis, risk for developing AML
  • Normocytic anemia with pancytopenia, negative LAP stain, decreased haptoglobin, increased Hb
  • Diagnose with flow cytometry
  • Treat with corticosteroids, Eculizumab to inhibit terminal complement activation
24
Q

Paroxysmal cold hemoglobinuria

A
  • Transient hemolytic anemia in kids with measles, mumps, flu, chicken pox; also associated with syphilis
  • IgG cold Ab (when moving from cold to warm environment, activates complement)
  • Fever, rigor, hemoglobinuria, back/leg/abd pain
  • May cause renal failure
  • Associated with Raynaud’s
  • Treat with plasma exchange to remove Ab
25
Q

G6PD deficiency

A
  • XLR
  • Deficient H2O2 neutralization; it oxidizes Hb, which precipitates in Heinz bodies, which lyse the RBC
  • Oxidant stresses include infection, drugs (ex. quinolones), fava beans
  • Sudden onset back pain, hemoglobinuria, jaundice
  • Normocytic anemia, bite cells
26
Q

Pyruvate kinase deficiency

A
  • Autosomal recessive
  • PEP isn’t converted to pyruvate in glycolysis
  • Low ATP damages RBC membrane; cell dehydrates, creating echinocytes
  • Jaundice, splenomegaly
  • Normocytic anemia
27
Q

Immune hemolytic anemias

A
  • Autoimmune, drug induced, or alloimmune
  • IgG hemolysis: spherocytes
  • Complement hemolysis
  • IgM hemolysis: RBC agglutination
  • Jaundice, hepatosplenomegaly, Raynaud’s (in cold AIHA)
  • Positive Coombs test
  • Normocytic anemia
  • Treat with corticosteroids, immune suppression, IV IgG (last resort; blocks from host IgG)
28
Q

Micro- and macroangiopathic hemolytic anemias

A
  • Often caused by aortic valve stenosis
  • Micro: thrombi cause RBC fragmentation, schistocytes
  • Macro: valve defect damages RBC
  • Normocytic anemia (possibly microcytic if longstanding)

My Pathology (12 decks)

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