Hematology I

Question 1

What is anemia?

Answer 1

decrease in RBCs, HCT, or Hb

Question 2

Erythropoiesis occurs in the:

Answer 2

bone marrow

Question 3

Erythropoietin comes from:

Answer 3

the juxtaglomerular (JG) cells of the kidney

Question 4

EPO is produced in response to:

Answer 4

decreased O2

increased androgens

Question 5

RBC lifespan:

Answer 5

120 days

Question 6

RBCs cleared by:

Answer 6

spleen, liver, bone marrow phagocytic cells

Question 7

Heme is degraded into:

Answer 7

bilirubin

Question 8

% of RBCs renewed daily:

Answer 8

0.5-1.5%

Question 9

Mechanisms of anemia:

Answer 9

Blood loss
Decreased absorption
Deficient erythropoeisis
Excessive hemolysis

Question 10

Hx risk factors for anemia:

Answer 10

Diet, menstruation, alcoholism, Rx drugs, cancer, COPD, marathon running, family hx of bleeding disorder.

Question 11

S/sx of anemia:

Answer 11

Weakness, fatigue, SOB, DOE, angina, syncope, drowsiness, seeing spots.
[vertigo, HA, tinnitus, pica, RLS, amenorrhea, menorrhagia, loss of libido, GI complaints]

Question 12

Signs of hemolysis:

Answer 12

jaundice, pruritis, dark urine (bilirubin, urobilinogen)

Question 13

Anemia PE:

Answer 13

orthostatis BP (supine, seated, standing)
Inspect conjunctiva, palms, mucus membranes, skin color
Inspect nails (blueness, ridges, spooning, clubbing)
Cardiovascular exam

Question 14

Microcytic causes:

Answer 14

Iron deficiency, thalassemia, Hb-synthesis defects, copper deficiency, zinc/lead poisoning, alcohol

Question 15

Macrocytic causes:

Answer 15

B12/folate deficiency, chemo, alcoholism, HIV anti-retrovirals, myelodysplastic disorder, impaired DNA synth

Question 16

Normocytic anemia causes:

Answer 16

blood loss, deficient EPO

Question 17

Most common anemia:

Answer 17

iron deficiency

Question 18

Iron deficiency anemia s/sx:

Answer 18

Mild - fatigue, HA, irritability, loss of stamina, pallor, difficult concentration.
Severe - pica, glossitis, cheilosis, spooning, tachycardia, dyspnea, RLS, glossal pain, xerostomia, alopecia

Question 19

Increases non-heme iron absorption:

Answer 19

ascorbic acid

Question 20

Daily iron requirement:

Answer 20

25mg/day (most from recycle/reabsorption)

Question 21

Iron storage forms/locations:

Answer 21

Hemosiderin - liver, marrow

Ferritin - liver, marrow, spleen, RBCs, serum

Question 22

Most frequent cause of iron deficiency anemia:

Answer 22

Men - chronic bleed (UC, colon cancer, PUD, ASA use)

Women - menstruation, repeated pregnancy

Question 23

Dx for iron deficient anemia:

Answer 23

Stool occult blood
Iron absorption test
CBC w/peripheral smear, serum iron, TIBC, serum ferritin

Question 24

Sideroblastic anemias are:

Answer 24

inadequate or abnormal utilization of marrow iron, with adequate stores. hereditary or 2° to drug/toxin (reversible)

Question 25

RBC appearance in sideroblastic anemia:

Answer 25

Polychromatophilic, stippled, targeted RBCs

Question 26

Dx for sideroblastic anemia:

Answer 26

CBC w/peripheral smear, iron, ferritin, BM bx, erythrocyte protoporphyrin, genetic studies

Question 27

Causes of anemia of chronic dz:

Answer 27

infection, inflammatory, neoplastic dz, trauma, heart failure, DM

Question 28

BM responsiveness to EPO mediated by cytokines:

Answer 28

IL-1 beta & TNF-alpha

Question 29

Hypoproliferative anemias are:

Answer 29

low marrow activity d/t lack of EPO or ability of BM to respond; seen in thyroid regulation disorders, panhypopituitarism

Question 30

Aplastic anemias are:

Answer 30

pancytopenia of all cell lines, or pure red cell aplasia

Question 31

Causes of aplastic anemias:

Answer 31

chemical exposure (drugs, pesticides, anti-cancer agents) infections (parvo, HIV, hepatitis, EBV, CMV) Genetic inability to clear toxins Fanconi's anemia (inherited)

Question 32

S/sx of aplastic anemia:

Answer 32

sudden acute onset or insidious, pallor, tachycardia, fatigue, dizziness Thrombocytopenia (petechiae, ecchymosis, bleeding gums, ocular fundi)

Question 33

Labs for aplastic anemia:

Answer 33

CBC w/peripheral smear, iron, reticulocytes, BM bx

Question 34

Myelophthisic anemia is:

Answer 34

marrow replaced by tumor, granuloma, fibrosis, or lipid storage dz

Question 35

S/sx of myelophthisic anemia:

Answer 35

Fatigue (early, severe), splenomegaly, night sweats, low fever, bone pain Could be asx

Question 36

Labs for myelophthisic anemia:

Answer 36

CBC w/peripheral smear, BM bx

Question 37

Macrocytic anemia is:

Answer 37

due to defective DNA synthesis

Question 38

causes of macrocytic anemia:

Answer 38

alcoholism, folate or B12 deficiency, chemo, hypothyroid, liver dz

Question 39

Anemia d/t B12/folate deficiency:

Answer 39

megaloblastic anemia

Question 40

S/sx of macrocytic anemia:

Answer 40

Usu none until severe Then neuro signs (stocking/glove neuropathy, dementia) Glossitis, diarrhea, muscle wasting

Question 41

Labs/dx for macrocytic anemia:

Answer 41

CBC w/peripheral smear, B12 (& PMN count), folate

Question 42

Causes of hemolytic anemia:

Answer 42

Intrinsic: abnormal RBC membranes RBC metabolism disorder Hemoglobinopathies Extrinsic: Hypersplenism Toxic exposure Autoimmune dz Mechanical injury (prosthetic valves, DIC, TTP) Infectious agents (malaria, C. Perfringens)

Question 43

S/sx of hemolytic anemia:

Answer 43

May be acute, chronic, or episodic Rapid onset pallor Acute hemolytic crisis (uncommon) - fever, chills, back/abd pain, prostration, shock Severe hemolysis: jaundice, splenomegaly, cholelithiasis

Question 44

Labs/dx for hemolytic anemia:

Answer 44

Peripheral smear - spherocytosis | Bilirubin, LDH, ALT

Question 45

Autoimmune hemolytic anemia is:

Answer 45

making antibodies to RBCs; may be severe & fatal

Question 46

Warm antibody hemolytic anemia:

Answer 46

``` >37°C more common in women spontaneous or from viral inf, immune deficiency, drugs, SLE, CLL, lymphoma primarily in the spleen mild splenomegaly ```

Question 47

Cold antibody hemolytic anemia:

Answer 47

<37°C commonly from infections (mycoplasma pneumoniae) 1/2 are idiopathic chronic primarily in the liver acrocyanosis, Raynauds, scleral icterus, splenomegaly

Question 48

Dx test for autoimmune hemolytic anemia:

Answer 48

Coombs - direct antiglobulin test

Question 49

Hereditary spherocytosis & elliptocytosis are:

Answer 49

congenital RBC membrane defects; autosomal dominant

Question 50

S/sx of hereditary -cytosis anemias:

Answer 50

``` Jaundice Splenomegaly Cholelithiasis Hepatomegaly (maybe) Anemia sx ```

Question 51

Stomatocytosis is:

Answer 51

Rare, congenital (severe) or acquired (EtOH) RBC membrane disorders, display increased fragility Show straight or rectangular central pallor

Question 52

Hypophasphatemia is:

Answer 52

d/t low phosphate, RBCs depleted of ATP -> rigid | small, spheroid RBCs

Question 53

G-6-P deficiency:

Answer 53

reduces energy available to maintain RBC membrane integrity d/t abnormal enzymes, genetic polymorphism, drug sensitivity x-linked 12% of African-American males fully express

Question 54

S/sx of G-6-P deficiency:

Answer 54

episodic sudden onset jaundice, pallor, dark urine, anemia sx may have back/abd pain may lead to renal failure

Question 55

Labs/dx of G-6-P deficiency:

Answer 55

G6P assay

Question 56

Sickle cell anemia:

Answer 56

Chronic hemolytic anemia; African descent; homozygous inheritance of Hb S gene (trait - heterozygous, varying expression)

Question 57

S/sx of sickle cell anemia:

Answer 57

``` painful bony crises renal damage punched out leg ulcers splenic infarcts aplastic crises anemia usually severe developmental delays avascular necrosis of femoral head abd pain with vomiting neurological disturbance splenomegaly ```

Question 58

Sever complications of sickle cell:

Answer 58

death by middle age, usu d/t infection, pulmonary emboli, vessel occlusion

Question 59

S/sx of sickle cell trait:

Answer 59

myalgia | hematuria during hypoxia

Question 60

Hemoglobin C dz:

Answer 60

genetic hemoglobinopathy; African descent; may be assoc with sickle cell

Question 61

S/sx of hemoglobin C dz:

Answer 61

bone pain splenomegaly jaundice (mild) anemia

Question 62

Thalassemia:

Answer 62

inherited defects in rate of synthesis of globin chains ineffective erythropoiesis & low RBC production Mediterranean, African, SE Asian, Pacific Islanders may occur with sickle cell anemia

Question 63

Thalassemia major:

Answer 63

homozygous most serious - beta thalassemia (autosomal dominant) usually fatal

Question 64

Thalassemia minor:

Answer 64

heterozygous milder dz may be asx

Question 65

S/sx of thalassemia:

Answer 65

``` heart failure failure to thrive splenomegaly jaundice leg ulcers gallstones thick zygomatic and cranial bones ```

Question 66

Leukopenia:

Answer 66

absolute decrease in number of WBCs circulating; may be pancytopenia or one line; <4000 c/mm

Question 67

Define neutropenia:

Answer 67

decrease in neutrophils (PMNs/granulocytes) | main defense against bacterial & fungal infections

Question 68

Neutropenia - normal resistance range:

Answer 68

>1500/mm whites | >1200/mm blacks

Question 69

Neutropenia - mild risk:

Answer 69

1000-1500/mm

Question 70

Neutropenia - moderate risk:

Answer 70

500-1000/mm

Question 71

Neutropenia - severe risk:

Answer 71

<500/mm

Question 72

Neutropenia - rapidly fatal range:

Answer 72

<200/mm

Question 73

S/sx of neutropenia:

Answer 73

sx only occur w/infection - fever (may be only sign) cellulitis, liver abscess, furunculosis, pneumonia, septicemia rash & LA if drug induced

Question 74

Intrinsic neutropenia:

Answer 74

due to defects in myeloid cells or precursors; rare; congenital, chronic idiopathic, benign

Question 75

Secondary neutropenia:

Answer 75

``` drug induced (chemo); unpredictable, idiosyncratic bone marrow infiltration - cancer, myelofibrosis hypersplenism infection, sepsis (childhood virus, HIV) alcoholism autoimmune - esp SLE folate/B12 deficiency leukemia transfusion ```

Question 76

Lymphocytopenia:

Answer 76

reduction in lymphocytes | <2yo

Question 77

Lymphocytes account for what % of WBCs?

Answer 77

20-40% | 30% - NLMEB

Question 78

Lymphocytes include:

Answer 78

T cells (75%) & B cells (25%) 65% of T cells are CD4

Question 79

Causes of lymphocytopenia:

Answer 79

infectious disease - TB, HIV, SARS, hep, EBV, CMV dietary deficiency Iatrogenic - immunosuppression, chemo, radiation systemic dz w/autoimmune - SLE, RA, sarcoid, cushing Other - EtOH, zinc deficiency

Question 80

S/sx of lymphocytopenia:

Answer 80

signs of primary illness absent/diminished tonsils or lymph nodes palpable LA if tumors are the cause hematologic dz - jaundice, pallor, petechiae, splenomegaly recurrent viral/fungal/parasitic infection

Question 81

Normal platelet count:

Answer 81

140-440K/uL

Question 82

Platelet lifespan:

Answer 82

7-10 days | destroyed by spleen

Question 83

Causes of thrombocytopenia:

Answer 83

``` failure of production increased sequestration, destruction, dilution quinine drugs liver dz DIC pregnancy, preeclampsia ITP (idiopathic thrombocytopenia purpura) HIV ```

Question 84

RBC size:

Answer 84

microcytic 95