Exam 3 Hematologic Slide Set 2 Flashcards

1
Q

Diagnosis of Autoimmune Hemolytic Anemia (AIHA) (Extrinsic Hemolytic Anemia)

A

Coomb’s & Indirect Coombs antiglobulin test

- detection of antibodies or complement is required to dx.

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2
Q

Warm Ab AIHA

A
  • Mot common form of AIHA
  • 50% idiopathic
  • 50% secondary to SLE or neoplastic dz (eg lymphoma)
  • IgG* is most common (IgA second)
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3
Q

Cold agglutinin AIHA

A
  • IgM Abs bind to RBCs at low temperatures (0-4 C)
  • IgM agglutinates & fixes Complement to the surface of the RBC
  • 15-30% of these anemias
  • Abs appear acutely during recovery from infections (Mono, HIV, Flu, etc) -> self-limiting & rarely causes clinical manifestations
  • Will occur chronically idiopathically or in response to some lymphoid neoplasms
  • > Raynaud phenomenon
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4
Q

Hemolytic Anemias (Extrinsic) resulting from Trauma

A

Causes: cardiac prosthesis, narrowing of microasculature (DIC), SLE, malignant HTN, TTP, HUS, cancer.

  • Mechanical injury to RBC
  • Peripheral smears show RBC fragments (schistocytes, burr cells, helmet cells
  • TTP and HUS are the only conditions that cause major clinical problems associated with hemolysis
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5
Q

Anemias of Diminished Erythropoiesis

A
  • Megaloblastic (deficiency) anemias
  • Iron Def Anemia
  • Anemia of chronic dz
  • anemia of renal failure
  • marrow stem cell failure (aplastic anemias)
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6
Q

Megaloblastic (macrocytic) anemias

A
  • B-12/folate deficiency (leads to inadequate thymidine) -> megaloblastic changes in all rapidly dividing cells -> b/c RNA synthesis is unaffected
  • Inadequate diet & alcoholism
  • Impaired DNA synthesis & distinctive morphological changes in the blood & bone marrow*
  • TPN/PPN, Azotemia/Uremia, Bacteremia/Septicemia?
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7
Q

Causes of Folic Acid Deficiency

A

-Alcoholism
-Phenytoin Sodium (anti-seizure medication)
-Anti-Metabolites (cancer drugs)
-HIV/AIDS drugs
(So… only things we do on purpose)

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8
Q

Morphological presentation in Megaloblastic anemia

A
  • RBC size & MCV increased
  • Anisocytosis (increased RDW)
  • Eosinophils & Neutrophils have hypersegmented nuclei
  • Low reticulocytes, some nucleated RBCs present in severe cases
  • Marrow is hyper cellular with increase in all myeloid precursors.
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9
Q

Pernicious anemia (cause, associations, populations affected, dx)

A
  • Lack of Intrinsic Factor leads to inadequate absorption of B-12
  • Assoc. w/gastric atrophy & achlorhydria, both often
  • Assoc. w/H pylori infection has NOT been proven yet
  • Pernicious anemia pts have 2-3x increase incidence of gastric carcinoma
  • Coexisting iron deficiency common b/c achlorhydria prevents getting ferric iron from food
  • Adult pernicious anemia most common in Celtic (English, Irish, Scottish) or Scandanavian origin
  • 10-20 cases/100,000 people/year
  • Does it all begin with auto reactive T-cell response that targets gastric mucosa???
  • *Gold standard dx = biopsy of gastric mucosa showing depleted parietal cells.
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10
Q

Antibodies found in pernicious anemia

A
  • Type 1 blocks binding of B-12 to IF
  • Type 2 blocks binding of IF/B-12 combo to its ileal receptor
  • Type 3 binds to proton pump, found in 50% of elderly patients with chronic gastritis
  • Anti-parietal cell Abs occur in 90% of pts with pernicious anemia and only 5% of healthy individuals
  • *Gold standard dx = biopsy of gastric mucosa showing depleted parietal cells.
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11
Q

Pernicious anemia morphology

A
  • Changes in marrow, alimentary tract, and NS
  • Alimentary tract = stomach & tongue: glossitis, intestinization (gastric epithelium replaced by goblet cells ie metaplasia)
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12
Q

General/other sx of B12 deficiency

A
  • NS sx in absence of anemia (more common in its taking folic acid or high-folate diet)
  • Older pts may present with dementia/Alzheimers sx: memory loss, irritability, personality changes
  • Megaloblastic madness: delusions, hallucinations, outbursts, paranoid schizophrenic ideation (myelin los in nerves of the posterior column)
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13
Q

Diagnostic features of B12 deficiency

A

-low serum RBC & B12, hgb, hct
-MCV elevated
WBC & platelets decreased
Increased homocysteine & methylmalonic acid (risk factors for atherosclerosis & thrombosis)
-Mild jaundice from ineffective erythropoesis & hemolysis
-**Inability to absorb oral B12 supplementation*

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14
Q

How does one become deficient in folate?

A
  • Poor diet (poor, elderly, those who don’t eat… green leafy vegetables, lemons, bananas, melons, yeast & liver!) or eat too much overcooked food.
  • *It’s not stored in the body
  • Interfering medications: dilantin, lithium, sulfasalazine, oral contraceptives
  • Also: alcoholism, hx of malabsorption, & pregnancy
  • US 4/100,000ppl have folate deficiency
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15
Q

Morphological presentation of folate deficiency

A
  • Megalocytes (circulating) & Megaloblasts (BM)
  • Hypersegmented neutrophils
  • Decreased serum folate or RBCs
  • Elevated serum homocysteine
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16
Q

Break it down - 3 major causes of folate deficiency

A
  • Decreased Intake: diet/absorption, alchoholism, drugs
  • Increased requirement: pregnancy, infancy, hemolytic anemias, cancer
  • Impaired use: folic acid antagonists, ie drugs
17
Q

General/other ramifications of Folate deficiency

A
Pregnant women
-premature birth
-neural tube defects
-placenta previa
Infants
-low birth wt
-spina bifida
-cleft lip
-cleft palate
18
Q

IDF

A
  • most common def in world: lack, absorption, requirement, blood loss
  • Common in developing countries -> vegetarian diet
  • Increased requirement in: infants, children, adolescents, menstruating women & pregnant women. Also when you have multiple/frequent pregnancies.
19
Q

Impaired iron absorption is found in these dz’s/conditions

A
  • Celiac
  • Other malabsorption causes (helpful!)
  • Chronic diarrhea
  • **And gastrectomy b/c that’s when the iron absorbing starts HCL + duodenum are really helpful
  • Chronic blood loss is the most common cause of IDF in the western world. (Gi, Gu, GYN!)
20
Q

IDA - iron deficiency anemia

A
  • microcytic/hypochromic
  • In: depleted stores, low serum iron, low serum ferritin, low transferrin saturation
  • Presentation: Koilonychia (concave nails), alopecia, atrophic changes to tongue, gastric mucosa, and intestine. Smear shows the lil’ cells & pencil/cigar cells or elliptocytes.
  • *disappearance of stainable iron from mononuclear phagocytic cells in the marrow is diagnostically significant.
21
Q

Dx of IDA

A
  • low ferritin
  • low iron
  • Elevated TIBC
22
Q

Anemia of Chronic Dz (ACD) - 3 C’s

A
  • Chronic infections (lung abscess, bacterial endocarditis, osteomyelitis)
  • Conective tissue dz (RA, SLE, Chron’s)
  • Cancer (Hodgkin lymphoma, lung/breast cancer and other CA)
  • Shows low iron AND low TIBC with normal or elevated ferritin!! B/c the little pin protein is doing stuff)
  • hypoproliferative anemia that is normocytic/normochromic, but if associated w/IDA it can be micro/hypo.
23
Q

Aplastic Anemias

A
  • The failure of pluripotent stem cells to produce RBCs, WBCs, and megakaryocytes
  • Morbidity/mortality usu from infection & bleeding
  • Sx: usual anemia & thrombocytopenia -> mucosal/gingival bleeding, petechial rash. Neutropenia -> infections
  • Bone marrow failure syndrome: PB pancytopenia or BM hypoplasia.
  • BM devoid of hematopoetic elemetnts, may show fat cells, fibrous stroma, or scattered foci of lymphocytes & plasma cells.
  • Result of T cells suppressing STEM cell fxn after exposure to drugs, infectious agents, or environmental toxins
  • 80% acquired, 20% congenital
24
Q

Acquired aplastic anemia (80%) causes

A
  • Infectious dz (EBV, HIV, hepatitis, etc)
  • Toxic exposure (radiation, benzene)
  • Drugs (chloramphenicol, sulfa, gold)
  • Idiopathic!
25
Q

The microcytic hypochromic anemias

A
  • IDA
  • Lead poisoning
  • Thalassemia
  • Hypothyroidism
  • ACD
26
Q

The macrocytic anemias

A
  • B12 def
  • Folic acid def
  • EOH abuse
  • Hyperthyroidism
27
Q

The normochromic normocytic anemias

A
  • acute hemorrhage/lysis
  • aplastic anemia
  • hypothyroid
  • ACD
  • vampires!