CLS 442 RBC Cards

iron deficiency anemia

• serum ferritin: decreased

• Serum iron: decreased/normal

• TIBC: increased

• Transferrin saturation: decreased

• FEP/ZPP: increased

• sTfR: increased

• Hg content of retics: decreased

• BM iron (Prussian blue reaction): no stainable iron

• Sideroblasts in BM: none

increased

TIBC level in IDA is ________ (increased/decreased/normal?)

increased

sTfR level in IDA is ________ (increased/decreased/normal?)

beta-thalassemia minor

• serum ferritin: increased/normal

• Serum iron: increased/normal

• TIBC: normal

• Transferrin saturation: increased/normal

• FEP/ZPP: normal

• sTfR: normal

• Hg content of retics: decreased

• BM iron (Prussian blue reaction): increased/normal

• Sideroblasts in BM: normal

anemia of chronic inflammation

• serum ferritin: increased/normal

• Serum iron: decreased

• TIBC: decreased

• Transferrin saturation: decreased/normal

• FEP/ZPP: increased

• sTfR: normal

• Hg content of retics: decreased

• BM iron (Prussian blue reaction): increased/normal

• Sideroblasts in BM: none/very few

• Other special tests: specific tests for inflammatory disorders or malignancy

sideroblastic anemia

• serum ferritin: increased

• Serum iron: increased

• TIBC: decreased/normal

• Transferrin saturation: increased

• FEP/ZPP: increased

• sTfR: normal

• Hg content of retics: normal

• BM iron (Prussian blue reaction): increased

• Sideroblasts in BM: increased (ring)

true

True or false: In IDA, ferritin levels begin to decrease in stage 1.

stage 2

At what stage of iron deficiency does TIBC begin to increase?

stage 3

At what stage of iron deficiency does the hemoglobin test decrease?

hepcidin

increased ______ inhibits iron absorption from intestines and iron release from macrophages/hepatocytes

normochromic, normocytic

rbc morphology in anemia of chronic inflammation

true

True or false: lead inhibits ALA dehydratase leading to accumulation of ALA

4.00-6.00 × 10^6/uL

ASCP reference index for RBC count

Vitamin B12 deficiency

serum MMA is increased in…..

pernicious anemia

an autoimmune disease in which an autoantibody is raised against IF (intrinsic factor) or gastric parietal cells

ineffective erythropoiesis

Why is the reticulocyte count low in megaloblastic anemia?

Schilling test

test used to distinguish cobalamin deficiency due to malabsorption, dietary deficiency, or absence of IF

Diphyllobothrium latum

What parasite may be responsible for a patient’s vitamin B12 deficiency?

pernicious anemia

An H. pylori infection may cause _______________ (type of anemia) by destroying parietal cells.

>7.5%

In stage one of the Schilling test, what result indicates normal B12 absorption?

12.0-18.0 g/dL

hemoglobin ASCP reference index

35-50%

ASCP reference range for HCT

76-100 fL

MCV ASCP reference range

26-34 pg

MCH ASCP reference range

32-36 g/dL

MCHC ASCP reference range

11.5-14.5%

RDW ASCP reference range

transferrin

Fe transport protein

ferritin

major Fe storage form

Hemosiderin

H2O insoluble Fe storage form (long-term)

Hemoglobin A (Hgb A)

Type of hemoglobin which comprises 97% of adult hemoglobin

HCT/RBC x 10

formula for MCV

HB/RBC x 10

formula for MCH

HB/HCT x 100

formula for MCHC

spherocytes

main abnormal RBC formed in extravascular (macrophage-mediated) hemolysis

schistocytes

main abnormal RBC formed in intravascular (fragmentation) hemolysis

anemia, splenomegaly, jaundice

triad of conditions seen in hereditary spherocytosis

hereditary spherocytosis

intrinsic RBC defect which involves mutations in proteins which maintain vertical attachements

ankyrin, spectrin

most of the protein mutations in hereditary spherocytosis are in ______ and _______

false

True or False: LDH is decreased in hereditary spherocytosis

hereditary spherocytosis

decrease in fluorescence in eosin-5’-maleimide binding test by flow cytometry is an expected result for which RBC defect?

hereditary elliptocytosis

rbc defects in horizontal interactions

paroxysmal nocturnal hemoglobin (PNH)

• rare chronic IV hemolytic anemia, acquired membrane defect

• RBCs lack CD55 and CD59

• absence of CD55 and CD59 renders RBCs susceptible to spontaneous lysis complement

CD55 and CD59 detection by flow cytometry

confirmatory test for PNH

G6PD deficiency

RBC deficiency which prevents the production of NADPH

oxidants would build up, leading to premature hemolysis

What happens if G6PD is missing in RBCs?

G6PD deficiency

• moderate to severe, usually normochromic/normocytic

• with severe variants: marked anisocytosis, poikilocytosis, spherocytosis, and schistocytosis

• Bite cells (not specific), Heinze bodies (denaturated Hb, only with supravital staining)

• Profound reticulocytosis (up to 30%)

• Very low serum haptoglobin, high indirect bilirubin, high LDH, increased plasma Hb (IV hemolysis)

PK deficiency

• __________ causes premature destruction of RBCs — without ___, depletion of ATP results in lack of membrane integrity

• Clinical presentation: anemia, jaundice, splenomegaly & gallstones (chronic hemolysis)

• neonates: severe anemia

• adults: severe to compensated

• PBS: anisocytosis, poikilocytosis, polychromasia, Burr cells

immune hemolytic anemia

• IgM mediated hemolysis can result in both extravascular and intravascular hemolysis

• IgG mediated hemolysis is predominantly extravascular → RBCs removed by macrophages in the spleen/liver

• DAT: positive

warm autoimmune hemolytic anemia (WAIHA)

• Immunoglobulin: IgG

• Optimum reactivity temperature of autoantibody: 37ºC

• Sensitization detected by DAT: IgG or IgG + complement

• Complement activation: variable

• Hemolysis: Extravascular primarily

• Autoantibody specificity: Panreactive

• Other laboratory findings: Polychromasia, spherocytes

cold agglutinin disease (CAD)

• Immunoglobulin: IgM

• Optimum reactivity temperature of autoantibody: 4ºC

• Sensitization detected by DAT: Complement

• Complement activation: Yes

• Hemolysis: Extravascular; some intravascular

• Autoantibody specificity: I (most), i (some), Pr (rare)

• Other laboratory findings: RBC agglutination may be present; hemoglobinuria

paroxysmal cold hemoglobin (PCH)

• Immunoglobulin: IgG

• Optimum reactivity temperature of autoantibody: 4ºC

• Sensitization detected by DAT: Complement

• Complement activation: Yes

• Hemolysis: Intravascular

• Autoantibody specificity: P (Donath-Landsteiner Ab)

• Other laboratory findings: Polychromasia, spherocytes, schistocytes, NRBCs, anisocytes, poikilocytosis; hemoglobinuria; Anti-P positive

lead poisoning (sideroblastic anemia)

urine ALA is high in _____

hemolytic uremia syndrome

• caused by bacteria that produce Shiga toxin

• the most common cause of disease is ingestion of improperly cooked meat especially in children

• the endothelial cell damage: activation of platelets, formation of platelet-fibrin thrombi, blockages in the microvasculature of the glomeruli, acute renal failure

thrombotic thrombocytopenic purpura (TTP)

a type of MAHA which is caused by a deficiency of ADAMTS13

hemoglobinuria (fragmentation hemolysis)

What does root beer-colored urine indicate?

false

True or false: In the heterozygous state of Hb E, there is a mean MCV of 55 fL

Hb SC

Which hemoglobinopathy has the following PBS findings:

• N/N anemia

• Increased polychromasia

• The reticulocyte count is 3% to 5%. Few sickle cells

• Targets, folded cells

• Intraerythrocytic blunt-ended crystals

  • “mitten cells” or “hand in glove”

A₂, E, O

Hb C migrates with __________ on alkaline electrophoresis

D, G

Hb S migrates with _____ on alkaline electrophoresis.

alkaline

______ electrophoresis must be done first and is enough to diagnose SS, AS, and thalassemia

HPLC

test involving separation and identification of both normal and variant hemoglobins is achieved based on varying ionic strength.

Hb Bart’s

Excess gamma (γ) chains form what type of Hb? (γ4)

True

True or false: Hb Bart’s has a very high oxygen affinity.

alpha

What type of thalassemia (beta or alpha) affects fetus and newborns?

beta-thalassemia silent carrier (heterozygous state)

type of beta-thalassemia with no hematologic abnormalities or clinical symptoms

beta-thalassemia minor (heterozygous state)

type of beta-thalassemia with mild hemolytic anemia, microcytic and hypochromic RBCs, and no clinical symptoms

beta-thalassemia major (homozygous or compound heterozygous state)

type of beta-thalassemia with severe hemolytic anemia, microcytic and hypochromic RBCs, severe clinical symptoms, and transfusion dependence

beta-thalassemia intermedia

type of beta-thalassemia with mild to moderate hemolytic anemia, microcytic and hypochromic RBCs, moderate clinical symptoms, and non-transfusion dependence

Hb H

hemoglobin which forms from excess beta chains as a result of the deletion of three alpha-globin genes

haptoglobin

glycoprotein which binds to free hemoglobin in blood circulation

Hb F

92-95% of the hemoglobin in beta-thal major patients is _____

Mentzer ratio

calculated index used to help with differentiation of IDA from beta-thalassemia minor

MCV/RBC count

calculation for Mentzer ratio

beta-thalassemia minor

If the Mentzer ratio is <13, the diagnosis favors _______.

iron deficiency anemia (IDA)

If the Mentzer ratio is >13, the diagnosis favors ______.

silent carrier

If only one alpha gene deletion is present, the patient is a ________ of alpha-thalassemia

alpha-thalassemia minor

a heterozygous alpha gene deletion (--/ɑɑ) results in what type of alpha-thalassemia

alpha-thalassemia minor

a homozygous alpha gene deletion (-ɑ/-ɑ) results in what type of alpha-thalassemia?

alpha-thalassemia intermedia/Hb H disease

deletion of three alpha chains (--/-ɑ) results in which type of alpha-thalassemia/what condition?

alpha-thalassemia intermedia

Hb H disease is also referred to as…?

hydrops fetalis (ɑ thal major)

Deletion of all alpha genes (- - / - -) results in what condition?

Hb Bart

Type of Hb which is present at 5-15% in newborn individuals with alpha-thalassemia minor.

alpha thalassemia

Are Hb H inclusions present as a result of alpha or beta thalassemia?

decreased

serum haptoglobin is _______ (decreased/normal/increased) in thalassemia major

G6PD deficiency

oxidizing drugs, infections, and fava beans are triggers of acute hemolytic anemia in what deficiency?