Rbc Hematology 2008 - Booth

Front 1

Composition of HgbF?

Back 1

α2γ2

Front 2

Composition of HgbA?

Back 2

α2β2

Front 3

At birth, about _____ HgbF and HgbA

Back 3

50-50
at 6 months it reaches normal adult levels

Front 4

Composition of HgbA2?

Back 4

α2δ2

Front 5

Anisocytosis = ?

Back 5

difference in RBC size

Front 6

Poikilocytosis = ?

Back 6

different RBC shapes

Front 7

MCV mean corpuscular volume = ?
Normal values?

Back 7

= HCT/RBC
Normal 80-100

Front 8

MCH mean corpuscular hemoglobin = ?

Back 8

HGB/RBC

Front 9

MCHC mean corpuscular hemoglobin concentration = ?

Back 9

MCH/MCV
>36 hyperchromic (commonly seen with spherocytes)
<32 hypochromic (common in iron deficiency)

Front 10

Half-life of RBC?

Back 10

120 days

Front 11

Anemia leads to tissue ______, results in increased ______ from kidney JG cells

Back 11

hypoxia
erythropoietin

Front 12

Corrected retic count (for anemia) =

Back 12

%retics x pt. Hct/normal Hct (use 45)

Front 13

Retic index (corrects for early bone marrow release) =

Back 13

corrected retic count/fudge factor

Front 14

Normal retic count =

Back 14

1%

Front 15

Retic index:
____is a good response to anemia. < ___ is bad response

Back 15

3-8
3

Front 16

Polychromasia =?

Back 16

young RBC, bluish cytoplasm

Front 17

Reticulocyte stain =?

Back 17

ribosomes/RNA, special stain

Front 18

Howell-Jolly body

Back 18

DNA
hemolytic anemia, megaloblastic anemia, splenectomy

Front 19

Pappenheimer body

Back 19

iron
hemolytic anemia, thalassemia, splenectomy

Front 20

Basophilic stippling

Back 20

RNA
pathological precipitation of ribosomes, thalassemia, lead poisoning

Front 21

Heinz bodies

Back 21

denatured hemoglobin
unstable hemoglobins, thalassemia, G6PD deficiency

Front 22

Target Cells

Back 22

macro = liver disease
normo = HgbC disease
micro = thalassemia

Front 23

Schistocytes

Back 23

microangiopathic hemolytic anemia (TTP, DIC, malignant hypertension, mechanical heart valve)

Front 24

Acanthocytes (irregular spikes)

Back 24

liver disease, abetalipoproteinemia

Front 25

Tear Drops

Back 25

thalassemia, myelofibrosis

Front 26

Echinocyte (spiny sea urchin or burr cells)

Back 26

uremia, pyruvate kinase deficiency

Front 27

Spherocyte

Back 27

hereditary, immune hemolytic anemia, transfusion reaction

Front 28

Sickle Cell

Back 28

SS, SC, S-thalassemia (NOT AS)

Front 29

Macrocytic anemia
What is deficient?
Conditions associated with it?

Back 29

B12 and folate deficiency, alcohol and liver disease, hypothyroidism

Front 30

Microcytic anemia
Seen with what conditions?

Back 30

Iron deficiency (chronic blood loss), thalassemia, sideroblastic anemia

Front 31

Seen in peripheral blood of macrocytic anemia.

Back 31

Peripheral blood “big bands” and hypersegmented neutrophils (>5 lobes)

Front 32

Made in stomach and needed for normal B12 absorption

Back 32

intrinsic factor

Front 33

Where is B12 absorbed?

Back 33

Terminal ileum absorption

Front 34

What causes B12 deficiency?

Back 34

Poor diet
Increased needs
Pregnancy, malignancy, hyperthyroidism
Stomach problems (no IF)
atrophic gastritis or pernicious anemia
gastrectomy
“in between” problems
bacterial overgrowth (blind loop syndrome)
fish tapeworm Diphyllobothrium latum
Terminal ileum problems
Crohn’s disease (terminal ileitis)
resection

Front 35

What is Pernicious anemia?

Back 35

a form of megaloblastic anemia due to vitamin B12 deficiency, caused by impaired absorption of vitamin B-12 due to the absence of intrinsic factor in the setting of atrophic gastritis, and more specifically of loss of gastric parietal cells.

Front 36

Where is folate absorbed?

Back 36

Jejunum absorption

Front 37

Seen in B12 deficiency but NOT in folate deficiency?

Back 37

neurologic complications

Front 38

Workup for macrocytic RBCs?

Back 38

Order serum B12 and serum folate
If B12 is the problem, order anti-intrinsic factor and anti-parietal cell antibodies

Front 39

The MOST common nutritional disorder in the world

Back 39

iron deficiency

Front 40

Site of iron uptake

Back 40

Duodenal uptake and regulation

Front 41

Etiology of iron deficiency

Back 41

poor diet
excess demand
*** chronic blood loss – GI or GU tumors, ulcers, angiodysplasia, esophageal varices, menstruation

Front 42

Iron deficiency signs and symptoms

Back 42

Signs and symptoms include koilonychia, Plummer-Vinson syndrome (microcytic hypochromic anemia, atrophic glossitis, esophageal webs)

Front 43

Low serum Fe
High TIBC
zero BM Fe

Back 43

Fe deficiency

Front 44

High serum Fe
Low TIBC
Increased BM Fe w/ ringed sideroblasts

Back 44

sideroblastic anemia

Front 45

Normal serum Fe
normal TIBC
Increased BM Fe

Back 45

Thalassemia

Front 46

Decreased serum Fe
Decreased TIBC
Increased BM Fe

Back 46

Chronic disease

Front 47

Seen in what disease?
Peripheral blood microcytic anemia, bizarre poikilocytosis with target cells

Back 47

thalassemia

Front 48

Beta thalassemia fun facts
gene mutation or gene deletion?

Back 48

2 beta genes (chromosome 11)
Mechanism = gene mutation
β β (normal genotype); β+ (decreased synthesis); β0 (no synthesis)
β thal major: both genes bad, severe anemia, transfusion dependent, increased HgbF
β thal minor: usually one bad gene, mild anemia, protects against falciparum malaria (also, sickle trait, G-6-PD deficiency), increased HgbA2
β thal intermedia

Front 49

Alpha thalassemia fun facts
gene mutation or gene deletion?

Back 49

4 genes (chromosome 16)
Mechanism = gene deletion
Lose 1gene, α-thal carrier
Lose 2 genes, α-thal trait, slight anemia
Lose 3 genes, Hgb H disease, live to adulthood, moderate anemia, Hgb H = β4 tetramer, Heinz bodies can be seen with brilliant cresyl blue
Lose 4 genes, Hgb Barts = γ4 tetramer, fatal, hydrops fetalis, die in utero or shortly after birth

Front 50

Composition of Hgb Barts? What disease is it associated with?

Back 50

γ4
Alpha thalassemia (Lost 4 genes, most severe kind of alpha thalassemia)
fatal, hydrops fetalis, die in utero or shortly after birth

Front 51

Complications of severe thalassemia:

Back 51

Bone marrow expansion and skeletal deformity
Pigmented gallstones
Hepatosplenomegaly
Secondary hemochromatosis (iron overload from transfusions, leads to heart failure, bronze diabetes, cirrhosis)

Front 52

Diagnosis of thalassemias?

Back 52

Family history; peripheral smear microcytic RBCs, many target cells
Hgb electrophoresis
Increased HgbF or A2 seen in β-thal.
Hgb H or Hgb Barts may be seen in α-thal.
Gene analysis can be done in utero

Front 53

Causes of normocytic anemias?

Back 53

Acute blood loss
Anemia of chronic disease = ↓ serum Fe, ↓ TIBC, BM Fe OK. Etiology ? liver (B12 or folate ?), kidney (↓ EPO)
Myelophthisic anemia: BM replacement by tumor (prostate, breast), fibrosis, granulomas, etc.
Aplastic anemia (BM failure): most are acquired: idiopathic, chemoRx, drugs (chloramphenicol- reversible dose-related, irreversible idiosyncratic); rarely inherited
Hemolytic anemias

Front 54

Intrinsic RBC defects

Back 54

Membrane (hereditary spherocytosis, PNH)
Enzymes (glucose-6-phosphate dehydrogenase deficiency)
Hgb structure (sickle cell anemia)

Front 55

Extrinsic cause of RBC destruction

Back 55

Antibodies (Coombs + hemolytic anemia)
Mechanical (e.g. heart valves)
Microangiopathic hemolytic anemia

Front 56

Dx clues for hemolytic anemias
Intravascular destruction

Back 56

LDH elevated, ↓ haptoglobin, hemoglobinemia, hemoglobinuria, hemosiderinuria, unconjugated bilirubinemia (jaundice)

Front 57

Dx clues for hemolytic anemias
Extravascular destruction

Back 57

Splenomegaly, jaundice, but not hemoglobinemia, not hemoglobinuria, not hemosiderinuria

Front 58

Hereditary spherocytosis fun facts:

Back 58

Spectrin or ankyrin deficiency
increased osmotic fragility
splenectomy can lessen the anemia

Front 59

PNH paroxysmal nocturnal hemoglobinuria fun facts

Back 59

Acquired defect is PIGA gene mutation leading to deficiency of glycosylphosphatidylinositol anchor protein
Results in lack of membrane CD55 (decay accelerating factor) and CD59 (membrane inhibitor of reactive lysis), leading to C`-mediated lysis of ALL cells (WBC, RBC, platelets = pancytopenia)

Front 60

Clinical triad of PNH

Back 60

hemolysis/pancytopenia/thrombosis

(watch out for venous thrombosis in these guys)

Front 61

Dx test for PNH

Back 61

flow cytometry to look for CD55/59

Front 62

G6PD fun facts

Back 62

Sex-linked, males, splenic destruction
Hemolytic episodes associated with drugs (Primaquine) or infection; also, fava beans (favism)
May protect against falciparum malaria

Front 63

2 things to look for periph blood smear of G6PD deficiency patients

Back 63

Heinz bodies and bite cells

Front 64

G6PD dx test

Back 64

quantitate enzyme levels! (don't do this during a hemolytic episode though)

Front 65

Genetic defect in sickle cell anemia?

Back 65

Sickle cell anemia: β chain #6 glutamate replaced by valine

Front 66

What can increase HgbF which has a protective effect against sickling?

Back 66

Hydroxyurea

Front 67

Acute cold agglutinans can be seen in which two infections?

Back 67

Mycoplasma pneumonia (anti-I specificity)
Infectious mononucleosis (anti-i specificity)

Front 68

Dx possibilities for schistocytes?

Back 68

DIC
HUS
TTP
Malignant hypertension
Vasculitides, e.g. SLE systemic lupus with vasculitis
Severe burns
Prosthetic heart valve
March hemoglobinuria

Front 69

Dx triad of HUS? Treatment?

Back 69

microangiopathic hemolytic anemia (schistocytes), thrombocytopenia, renal failure.
supportive, dialysis as necessary, do NOT give antimicrobials or antidiarrheals

Front 70

Dx pentad of TTP?

Back 70

like HUS plus fever and neurologic signs (confusion, seizure, etc.);

Front 71

Most common etiology of TTP?

Back 71

autoimmune disease, antibodies to von Willebrand cleaving enzyme (ADAMTS 13).
clopidogrel (Plavix) is a frequent etiology.

Front 72

Some good screening tests for porphyria?

Back 72

Urine PBG is a good screening test during acute attacks
Fluorescent urine (with a black light or Wood’s lamp) is a good clue in babies

Front 73

Polycythemia
Reactive/relative, associated with ??

Back 73

dehydration, Gaisbock stress syndrome

Front 74

Primary polycythemia rubra vera (decreased EPO)

Back 74

a chronic myeloproliferative disease; JAK 2 mutation in 90%
Acquired clonal stem cell disorder, elderly patients
RBC’s growing out of control, so EPO is low or non-detectable
Splenomegaly
Increased risk acute leukemia; burnout stage myelofibrosis of marrow

Front 75

Secondary polycythemia (increased EPO)

Back 75

Seen in lung disease/smokers, heart disease
EPO-producing tumors: renal cell Ca, cerebellar hemangioblastoma