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Flashcards in Hematology Deck (56):
0

erythropoietin regulates

red cell production

1

reticulocytes are

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

2

mature RBC

*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.

3

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

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

4

porphyrin ring from RBC breakdown is metabolized into

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

5

anemia with MCV<80

microcytic: iron def, chronic dz,thalassemia, sideroblastic

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

renal failure
hypothyroidism
chronic disease
aplastic anemia

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anemia with MCV 81-99 and increased reticulocyte count think

hemolysis
blood loss

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anemia with MCV>100 think

megaloblastic (bone marrow)
non megaloblastic

9

clinical features of iron def anemia

*50% Pica->ice,clay,starch,potatoe chips cravings
*cheilosis -fissures at corner of mouth & koilonychia-> severe iron def.

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causes of iron def anemia

*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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Dx iron def anemia

low iron, low transferrin sat. (100

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Tx iron def anemia

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

13

RBC fragments (schistocytes) mean

*microangiopathic hemolytic anemia (TTP,HUS,HELLP,DIC,and occ vasculitis)
*severe burns
*valve hemolysis

14

spherocytes mean

*autoimmune hemolytic anemia
*hereditary spherocytosis

15

target cells can be seen in

*significant liver disease
*thalassemia and other hemoglobinopathies

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teardrop cells seen in

*classic seenin myelofibrosis and other infiltrating bone marrow process
*thalassemia

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Burr cells seen in

uremic patients

18

Spur cells are seen in

liver diseases

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Howell-Jolly bodies seen in

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

20

hypersegmented PMN's seen in

megaloblastic anemia
pernicious anemia
B12/folate def

21

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?

hemolytic anemia
do coombs test and peripheral blood smear

22

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

megaloblastic anemia

23

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

colonoscopy

24

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

celiac disease, best dx: IgA endosymial antibody

25

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

multiple myeloma. do urine and serum electrophoresis

26

iron def anemia RDW and RBC count

increased RDW
decreased RBC count

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thalasemia minor RBC count and RDW

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

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iron def anemia see

transferrin sat <30

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anemia of chronic disease

*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

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most common genetic disorder worldwide

thalassemia

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thalassemia is a defective

production of alpha or beta chains of Hb.
see microcytic hypochromic anemia and incr RBC count

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Beta thalassemia major (colleys anemia)

*severe hemolytic anemia from childhood
*hepatosplenomegaly
*peripheral smear:target cells, tear drop cells, nucleated RBC's

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Tx of beta thalassemia major

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

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complications of beta thalassemia major

*hemosiderosis=> cardiomyopathy, mult hormone deficiencies
*incr risk of venous and arterial thrombus due to RBC membrane abnormality

35

Hemoglobin H disease (Beta 4 Hb)

*Hgb range 6-10
*microcytic hypochromic anemia
*splenomegaly
*Heinz bodies on RBC's

36


Sideroblastic anemia

***ring sideroblasts in bone marrow

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causes of sideroblastic anemia

*chronic inflammation
*malignancy
*pyridoxine defic., copper deficiency
*INH, alcohol, lead poisoning

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tx sideroblastic anemia

pyridoxine, erythropoietin

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causes of megaloblastic macrocytic anemia (MCV>100)

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

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

alcoholism
liver dz,hemolysis
blood loss
hypothyroidism
AZT

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pernicious anemia

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

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complications of pernicious anemia

*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

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

measure methylmalonic acid, if elevated confirms B12 deficiency

44

B12 anemia can be corrected by folic acid but may cause

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

45

Tx of pernicious anemia

B12 1000-2000ug/d

46

causes of folic acid deficiency:
*inadequate nutrition:alcoholism, malnutrition
*increased requirement: chronic hemolysis, pregnancy,psoriasis
*malabsorption:sprue,alcohol, phenytoin, barbiturates

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

47

myelodysplastic syndrome is

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

48

myelodysplastic syndrome see

*macrocytic anemia w normal B12 and folate
*bleeding or infection d/t abnl platelet and granulocytic fxn

49

Peripheral smear in myelodysplastic syndrome

Pelgar huet anomaly of neutrophils (1-2 lobes)
immature WBC's with cytoplasmic hypogranulation

50

bone marrow in myelodysplastic syndrome

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

51

Tx myelodysplastic syndrome

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

52

#1 genetic disorder in the white population

hemochromatosis

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cause of hemochromatosis

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

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diagnosis of hemochromatosis

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

55

Manifestations of Hemochromatosis

*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