Hematology

Question 0

reticulocytes are

Answer 0

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

Question 1

erythropoietin regulates

Answer 1

red cell production

Question 2

mature RBC

Answer 2

• 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.

Question 3

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

Answer 3

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

Question 4

porphyrin ring from RBC breakdown is metabolized into

Answer 4

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

Question 5

anemia with MCV<80

Answer 5

microcytic: iron def, chronic dz,thalassemia, sideroblastic

Question 6

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

Answer 6

renal failure
hypothyroidism
chronic disease
aplastic anemia

Question 7

anemia with MCV 81-99 and increased reticulocyte count think

Answer 7

hemolysis

blood loss

Question 8

anemia with MCV>100 think

Answer 8

megaloblastic (bone marrow)

non megaloblastic

Question 9

clinical features of iron def anemia

Answer 9

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

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

Question 10

causes of iron def anemia

Answer 10

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

Question 11

Dx iron def anemia

Answer 11

low iron, low transferrin sat. (100

Question 12

Tx iron def anemia

Answer 12

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

Question 13

RBC fragments (schistocytes) mean

Answer 13

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

Question 14

spherocytes mean

Answer 14

• autoimmune hemolytic anemia

* hereditary spherocytosis

Question 15

target cells can be seen in

Answer 15

• significant liver disease

* thalassemia and other hemoglobinopathies

Question 16

teardrop cells seen in

Answer 16

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

Question 17

Burr cells seen in

Answer 17

uremic patients

Question 18

Spur cells are seen in

Answer 18

liver diseases

Question 19

Howell-Jolly bodies seen in

Answer 19

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

Question 20

hypersegmented PMN’s seen in

Answer 20

megaloblastic anemia
pernicious anemia
B12/folate def

Question 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?

Answer 21

hemolytic anemia

do coombs test and peripheral blood smear

Question 22

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

Answer 22

megaloblastic anemia

Question 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?

Answer 23

colonoscopy

Question 24

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

Answer 24

celiac disease, best dx: IgA endosymial antibody

Question 25

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

Answer 25

multiple myeloma. do urine and serum electrophoresis

Question 26

iron def anemia RDW and RBC count

Answer 26

increased RDW | decreased RBC count

Question 27

thalasemia minor RBC count and RDW

Answer 27

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

Question 28

iron def anemia see

Answer 28

transferrin sat <30

Question 29

anemia of chronic disease

Answer 29

* 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

Question 30

most common genetic disorder worldwide

Answer 30

thalassemia

Question 31

thalassemia is a defective

Answer 31

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

Question 32

Beta thalassemia major (colleys anemia)

Answer 32

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

Question 33

Tx of beta thalassemia major

Answer 33

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

Question 34

complications of beta thalassemia major

Answer 34

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

Question 35

Hemoglobin H disease (Beta 4 Hb)

Answer 35

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

Question 36

Sideroblastic anemia

Answer 36

***ring sideroblasts in bone marrow

Question 37

causes of sideroblastic anemia

Answer 37

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

Question 38

tx sideroblastic anemia

Answer 38

pyridoxine, erythropoietin

Question 39

causes of megaloblastic macrocytic anemia (MCV>100)

Answer 39

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

Question 40

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

Answer 40

``` alcoholism liver dz,hemolysis blood loss hypothyroidism AZT ```

Question 41

pernicious anemia

Answer 41

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

Question 42

complications of pernicious anemia

Answer 42

*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

Question 43

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

Answer 43

measure methylmalonic acid, if elevated confirms B12 deficiency

Question 44

B12 anemia can be corrected by folic acid but may cause

Answer 44

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

Question 45

Tx of pernicious anemia

Answer 45

B12 1000-2000ug/d

Question 46

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

Answer 46

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

Question 47

myelodysplastic syndrome is

Answer 47

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

Question 48

myelodysplastic syndrome see

Answer 48

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

Question 49

Peripheral smear in myelodysplastic syndrome

Answer 49

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

Question 50

bone marrow in myelodysplastic syndrome

Answer 50

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

Question 51

Tx myelodysplastic syndrome

Answer 51

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

Question 52

#1 genetic disorder in the white population

Answer 52

hemochromatosis

Question 53

cause of hemochromatosis

Answer 53

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

Question 54

diagnosis of hemochromatosis

Answer 54

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

Question 55

Manifestations of Hemochromatosis

Answer 55

* 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