Hematology Flashcards

0
Q

reticulocytes are

A

immature RBC that have lost their nucleus but retain their RNA, can be stained and counted => reticulocyte count

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1
Q

erythropoietin regulates

A

red cell production

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2
Q

mature RBC

A
  • contain no RNA
  • live approx 120 days
  • pass repeatedly thru spleen where damaged/old ones are ingested by macrophages, hemoglobin breaks down to heme and porphyrin ring.
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3
Q

heme releases iron (and iron absorbed from GI tract)is transported by transferrin to

A

bone marrow and other tissues and stored as ferritin and hemosiderin.

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4
Q

porphyrin ring from RBC breakdown is metabolized into

A

unconjugated (indirect) bilirubin> binds to albumin>goes to liver where it is conjugated.

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5
Q

anemia with MCV<80

A

microcytic: iron def, chronic dz,thalassemia, sideroblastic

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6
Q

anemia with MCV 81-99 with decreased reticulocyte count, think

A

renal failure
hypothyroidism
chronic disease
aplastic anemia

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7
Q

anemia with MCV 81-99 and increased reticulocyte count think

A

hemolysis

blood loss

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8
Q

anemia with MCV>100 think

A

megaloblastic (bone marrow)

non megaloblastic

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9
Q

clinical features of iron def anemia

A
  • 50% Pica->ice,clay,starch,potatoe chips cravings

* cheilosis -fissures at corner of mouth & koilonychia-> severe iron def.

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10
Q

causes of iron def anemia

A
  • increase demand for iron-rapid growth, preg,erythropoietin Tx
  • blood loss
  • decreased iron intake or absorption-poor diet, malabsorption (celiac, crohns) postgastrectomy, inflammation
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11
Q

Dx iron def anemia

A

low iron, low transferrin sat. (100

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12
Q

Tx iron def anemia

A

oral iron - 300mg of elemental iron/day-iron BID until hgb is 10, then daily
parenteral iron- if cant tolerate oral or if malabsorption. small risk of anaphylaxis

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13
Q

RBC fragments (schistocytes) mean

A
  • microangiopathic hemolytic anemia (TTP,HUS,HELLP,DIC,and occ vasculitis)
  • severe burns
  • valve hemolysis
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14
Q

spherocytes mean

A
  • autoimmune hemolytic anemia

* hereditary spherocytosis

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15
Q

target cells can be seen in

A
  • significant liver disease

* thalassemia and other hemoglobinopathies

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16
Q

teardrop cells seen in

A
  • classic seenin myelofibrosis and other infiltrating bone marrow process
  • thalassemia
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17
Q

Burr cells seen in

A

uremic patients

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18
Q

Spur cells are seen in

A

liver diseases

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19
Q

Howell-Jolly bodies seen in

A

splenectomy or functional asplenia
* H-J bodies are due to fragmentation of the nucleus causing small black pellets. this occurs in normal patient but spleen removes them

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20
Q

hypersegmented PMN’s seen in

A

megaloblastic anemia
pernicious anemia
B12/folate def

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21
Q

Anemia with a retic count 130,000, Incr LDH & bilirubin. no evidence of blood loss. what is cause and what test would you do next?

A

hemolytic anemia

do coombs test and peripheral blood smear

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22
Q

anemia, incr LDH (intramedullary destr of cells). incr bilirubin, MCV 120,retic count 20,000, smear with PMN’s with >5 lobes. Dx?

A

megaloblastic anemia

23
Q

70 yr old with anemia and decreased MCV, decr Fe, incr TIBC, decr transferrin sat of 10%, and low ferritin. what to do next?

A

colonoscopy

24
Q

30 yr old with chronic diarrhea and iron def. anemia, likely Dx

A

celiac disease, best dx: IgA endosymial antibody

25
Q

anemia, LBP,incr Ca+, compression fracture of vertebra and incr sedrate. ?Dx

A

multiple myeloma. do urine and serum electrophoresis

26
Q

iron def anemia RDW and RBC count

A

increased RDW

decreased RBC count

27
Q

thalasemia minor RBC count and RDW

A

RDW normal
increased RBC count
test Hgb electrophoresis , if increased its thal trait

28
Q

iron def anemia see

A

transferrin sat <30

29
Q

anemia of chronic disease

A
  • transferrin sat 100= anemia chronic dz
  • serum ferritin 30-100, check ratio of soluble transferrin to log serum ferritin: if >2, both iron def and chronic dz, if <1, its anemia of chronic dz
30
Q

most common genetic disorder worldwide

A

thalassemia

31
Q

thalassemia is a defective

A

production of alpha or beta chains of Hb.

see microcytic hypochromic anemia and incr RBC count

32
Q

Beta thalassemia major (colleys anemia)

A
  • severe hemolytic anemia from childhood
  • hepatosplenomegaly
  • peripheral smear:target cells, tear drop cells, nucleated RBC’s
33
Q

Tx of beta thalassemia major

A

blood transfusion to maintain Hgb -10gm
desferoxamine to chelate iron
vitamin E
acetylcysteine

34
Q

complications of beta thalassemia major

A
  • hemosiderosis=> cardiomyopathy, mult hormone deficiencies

* incr risk of venous and arterial thrombus due to RBC membrane abnormality

35
Q

Hemoglobin H disease (Beta 4 Hb)

A
  • Hgb range 6-10
  • microcytic hypochromic anemia
  • splenomegaly
  • Heinz bodies on RBC’s
36
Q

Sideroblastic anemia

A

***ring sideroblasts in bone marrow

37
Q

causes of sideroblastic anemia

A
  • chronic inflammation
  • malignancy
  • pyridoxine defic., copper deficiency
  • INH, alcohol, lead poisoning
38
Q

tx sideroblastic anemia

A

pyridoxine, erythropoietin

39
Q

causes of megaloblastic macrocytic anemia (MCV>100)

A

B12deficiency, folate deficiency
myelodysplastic syndrome
S/E of anticancer drug-azathioprine

40
Q

causes of non-megaloblastic macrocytic anemia (MCV>100)

A
alcoholism
liver dz,hemolysis
blood loss
hypothyroidism
AZT
41
Q

pernicious anemia

A

B12 deficiency due to autoimmune destruction of parietal cells that produce intrinsic factor

42
Q

complications of pernicious anemia

A

*neuro:peripheral neuropathy, ataxia,spastic paraplegia, loss of vibratory and position sense, dementia, psychosis
GI:anorexia, wt loss,diarrhea,malabsorption, gastric ca
hypothyroidism, other autoimmune disorders

43
Q

what to do if suspect B12 def and B12 level is borderline?

A

measure methylmalonic acid, if elevated confirms B12 deficiency

44
Q

B12 anemia can be corrected by folic acid but may cause

A

worsening of neuropsych symptoms. MUST exclude B12 deficiency in pt with folic acid def before giving folic acid

45
Q

Tx of pernicious anemia

A

B12 1000-2000ug/d

46
Q

causes of folic acid deficiency:

  • inadequate nutrition:alcoholism, malnutrition
  • increased requirement: chronic hemolysis, pregnancy,psoriasis
  • malabsorption:sprue,alcohol, phenytoin, barbiturates
A

*inhibitors of dihydrofolate reductase:methotrexate, pyrimethamine, triamterene, pentamidine, trimethoprim, alcohol

47
Q

myelodysplastic syndrome is

A

a stem cell disorder leading to varying degrees of cytopenias affecting one or more cell lines

48
Q

myelodysplastic syndrome see

A
  • macrocytic anemia w normal B12 and folate

* bleeding or infection d/t abnl platelet and granulocytic fxn

49
Q

Peripheral smear in myelodysplastic syndrome

A

Pelgar huet anomaly of neutrophils (1-2 lobes)

immature WBC’s with cytoplasmic hypogranulation

50
Q

bone marrow in myelodysplastic syndrome

A

cellular marrow w cytological atypia, megaloblasts, ring sideroblasts
*blasts (5-19%)- the higher the blasts, worst prognosis

51
Q

Tx myelodysplastic syndrome

A
  • erythropoietin + G CSF, azacitidine, blood transfusions
  • lenalidomide ffor 5q syndrome: anemia, high platelet count
  • allogenic bone marrow transplant
52
Q

1 genetic disorder in the white population

A

hemochromatosis

53
Q

cause of hemochromatosis

A

HFE gene mutation (C 282Y or H 63D)

54
Q

diagnosis of hemochromatosis

A

transferrin saturation >50% male, >45% female , then do gene test. also ferritin is done too.

55
Q

Manifestations of Hemochromatosis

A
  • skin pigmentation, cardiomyopathy
  • Liver: abn LFT’s, fibrosis, cirrhosis, carcinoma
  • diabetes, hypopituitarism
  • arthritis-exp 2 & 3rd metacarpophalangeal most common joints
  • infection:vibrio, listerria,yersinia, salmonella, Kleb. E Coli,Mucor