Aplastic anemia - 4 secondary causes

- Chemicals (benzene, arsenic, insecticides, weed killers) - Drugs (chloramphenical) - Radiation (long term, low dose) - Infections, esp. chronic ( Hepatitis C, EBV, CMV, HIV)

Aplastic anemia - CBC

- WBC \< 1500/cumm - ↓ RBC - Hb \< 7 g/dL - ↓ Hct - ↓ PLT

Aplastic anemia - Treatment

- Take away offending agent, if applicable - "support" therapy as needed (antibiotics, blood products esp. plts, use of growth factors) - immunosuppressive therapy to stimulate BM - Only cure is BM transplant

Sickle cell anemia - RBC morphology

- Poik → "targets plus" sickles, schistos, spheres - Polychromasia (increased retic count) - RBC inclusions → H-J bodies, Pappenhemier - nRBCs

Sickle cell anemia - Treatment

- Adequate hydration - Pain relief from crises (morphine) - Antibiotics - Blood transfusions - Hydroxyurea to increase hemoglobin F...relieve sickling

N/N Anemias Flashcards by Liz Haywood

Aplastic anemia

Common cause

Idiopathic (unknown cause)

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Aplastic anemia

4 secondary causes

Chemicals (benzene, arsenic, insecticides, weed killers)
Drugs (chloramphenical)
Radiation (long term, low dose)
Infections, esp. chronic ( Hepatitis C, EBV, CMV, HIV)

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Aplastic anemia

Name of most comon congenital disorder associated w/ it

Fanconi’s anemia

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Aplastic anemia

BM cellularity

Hypocellular

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Aplastic anemia

WBC < 1500/cumm
↓ RBC
Hb < 7 g/dL
↓ Hct
↓ PLT

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Aplastic anemia

Characteristic RBC morphology

None, just few present

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Aplastic anemia

Retic count

Decreased to absent

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Aplastic anemia

Treatment

Take away offending agent, if applicable
“support” therapy as needed (antibiotics, blood products esp. plts, use of growth factors)
immunosuppressive therapy to stimulate BM
Only cure is BM transplant

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Type of poik found in most every hemoglobinopathy

Sickle cells

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Amino acid substitution found in sickle cell anemia

Glutamic acid replaced by valine

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3 factors contributing to sickling process

Hypoxia
Acidosis
Dehydration

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Sickle cell anemia

Cause of “painful crises”

Tissue damage from hypoxia

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Sickle cell anemia

Cause of “acute chest syndrome”

Infarction of organs

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Sickle cell anemia

Cause of high risk of infections

Splenomegaly to autosplenectomy

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Sickle cell anemia

Inheritance

Abnormal gene from both parents

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Sickle cell anemia

Hemoglobin nomenclature

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Sickle cell anemia

Solubility (Sickledex) results

Hemoglobin S is insoluble → precipitates in solution = turbid (+)

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Sickle cell anemia

Hemoglobin electrophoresis results

S > F (no A)

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Sickle cell anemia

RBC morphology

Poik → “targets plus” sickles, schistos, spheres
Polychromasia (increased retic count)
RBC inclusions → H-J bodies, Pappenhemier
nRBCs

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Sickle cell anemia

Treatment

Adequate hydration
Pain relief from crises (morphine)
Antibiotics
Blood transfusions
Hydroxyurea to increase hemoglobin F…relieve sickling

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Sickle cell trait

Inheritance

Abnormal gene from one parent

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Sickle cell trait

Hemoglobin nomenclature

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Sickle cell trait

Solubility (Sickledex) results

Hemoglobin A is soluble = solution remains clear (-)

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Sickle cell trait

Hemoglobin electrophoresis results

A > S

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Sickle cell trait - RBC morphology

- Slight targets - No sickles (treatment may occur under severe hypoxic states)

Sickle cell trait - Treatment

No treatment necessary

Sickledex solubility (screening) test - Principle

Blood added to buffered solution of reducing agent

Sickledex solubility (screening) test - Reducing agent

Sodium dithionite or sodium metabisulfite

Sickledex solubility (screening) test - Causes for false positive results

- Proteinemia - \> 18 g/dL hemoglobin - Other sickling hemoglobins

Sickledex solubility (screening) test - Causes for false negative results

- Testing a newborn - \< 7 g/dL hemoglobin - Multiple transfusions

Amino acid substitution found in hemoglobin C disease

Glutamic acid substituted w/ lysine

Hemoglobin C disease - Clinical presentation

Mildly hemolytic anemia

Hemoglobin C disease - Hemoglobin nomenclature

Hemoglobin C disease - Hemoglobin electrophoresis results

100% C (no A)

Hemoglobin C disease - RBC morphology

- Poik → "targets plus" C crystals - Polychromasia (increased retic count)

Hemoglobin C trait - Clinical presentation

Asymptomatic

Hemoglobin C trait - Hemoglobin nomenclature

Hemoglobin C trait - Hemoglobin electrophoresis results

A \> C

Hemoglobin C trait - RBC morphology

Targets only

Hemoglobin SC disease - Inheritance

- Lysine substitution (C) from one parent - Valine substitution (S) from the other

Hemoglobin SC disease - Clinical presentation

Less severe than sickle cell disease (SS), but still mild-moderately (severe) hemolytic anemia w/ painful crises

Hemoglobin SC disease - Hemoglobin electrophoresis results

S = C

Hemoglobin SC disease - RBC morphology

- Poik → "targets plus" sickles, C crystals, and SC crystals - Polychromasia (increased retic count) - RBC inclusions (H-J bodies, Pappenheimer) - nRBCs

Sickle cell ß thal - Inheritance

- Valine substitution from one parent - ß⁰ or ß⁺ from the other

Sickle cell ß thal - Clinical presentation (sickle ß⁰ thal vs. sickle ß⁺thal)

- ß⁰ → severe hemolytic anemia - ß⁺ → mild to moderate hemolytic anemia

Sickle cell ß thal - Hemoglobin electrophoresis results (sickle ß0 thal vs. sickle ß+ thal)

- ß⁰ → S \> F (↑) \> A2 (↑) w/ no A - ß⁺ → S \> A \> F (↑) \> A2 (↑)

Sickle cell ß thal - RBC morphology (sickle ß0 thal vs. sickle ß+ thal)

- ß⁰ and ß⁺ → "targets plus" sickles (sickles may be absent w/ mild ß⁺), schistos, spherocytes, nRBCs

2 ways that hemoglobin D may be differentiated from hemoglobin S since they both migrate to the same point on cellulose acetate electrophoresis

- Solubility testing (hemoglobin D will be negative) - Citrate (acid) electrophoresis

World's third most common abnormal hemoglobin (behind Hb S and Hb C) and indicate the geographic ara in which it commonly occurs

- Hemoglobin E - Common in SE Asia

Physiological mechanism for predominant type of poik found in hereditary spherocytosis

↓ spectrin causes ↑ permeability of sodium into cell

Physiological mechanism for predominant type of poik found in hereditary elliptocytosis

↓ cholesterol in cell membrane causes Hb to polarize to opposite ends

Physiological mechanism for predominant type of poik found in hereditary stomatocytosis

Defect in NaK-ATPase pump → ↓ Na⁺ in and ↑ K⁺ out → abnormal slit-like pallor

Hereditary spherocytosis - Clinical presentation

Anemia, jaundice, splenomegaly

Hereditary spherocytosis - RBC indices

- ~12 g/dL hemoglobin - MCHC = 36-38%

Hereditary spherocytosis - RBC morphology

- Variable # in spheres - Polychromasia (increased retic count)

Osmotic fragility test - Principle

Blood is added to series of hypotonic salt solutions, beginning and completion of hemolysis are noted

Osmotic fragility - Conditions that show "increased osmotic fragility"

Hereditary spherocytosis

Osmotic fragility test - Conditions that show "decreased osmotic fragility"

- Thalassemia - Sickle cell anemia - Any hypochromic anemia

Osmotic fragility test - Conditions that show "decreased resistance to hemolysis"

Hereditary spherocytosis

Osmotic fragility test - Conditions that show "increased resistance to hemolysis"

- Thalassemia - Sickle cell anemia - Any hypochromic anemia

Osmotic fragility test - NaCl concentration when hemolysis should begin (in a normal person)

0.45-0.5% NaCl

Osmotic fragility test - NaCl concentration when hemolysis should be completed (in a normal person)

0.3-0.35% NaCl

Type of poik that demonstrates greatest resistance to hemolysis

Target cells, hypochromic sickles

Expected results that occur w/ any hemolytic anemia - Plasma haptoglobin

Decreased

Expected results that occur w/ any hemolytic anemia - Retic count

Increased

Expected results that occur w/ any hemolytic anemia - Serum bilirubin

Increased

G-6-PD deficiency - Result of deficient enzyme - Type of poik or inclusion bodies present - Triggering factor

- Glutathione is not reduced - Heinz bodies, bite cells - Administration of new drug, infection, ingestion of fava beans, ingestion of moth balls

PK deficiency - Result of deficient enzyme - Type of poik or inclusion bodies present - Triggering factor

- Decreased capacity to generate ATP - Burr cells - Triggering factors?

Methemoglobin reductase deficiency - Result of deficient enzyme - Type of poik or inclusion bodies present - Triggering factor

- Increased levels of methemoglobin - Poik? - Triggering factors?

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Etiology

- Causes cells, esp. RBCs, to be more sensitive than normal to lytic action of complement (in an acid environment)

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Clinical presentation

- "sleep-induced hemolytic anemia" → bloody first morning urine, clears throughout day

Paroxysmal Nocturnal Hemoglobinuria (PNH) - CBC

Pancytopenia

Paroxysmal Nocturnal Hemoglobinuria (PNH) - RBC morphology

None

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Ham's test results

Positive

Principle of Ham's test

Due to intrinsic membrane defect, PNH red cells are more sensitive to lysis by complement. A pH of 6.7-7.0 and 37°C provide optimum conditions for complement activation via alternate pathway and subsequent lysis fo PNH red cells

3 conditions that may cause an alloimmune hemolytic anemia to develop

- Transfusions - Pregnancy (HDFN) - Organ transplantation

Abnormality in immune system whereby the ability for self-recognition of an individual's own red cell Ags is lost

Autoimmune hemolytic anemia

Abs produced by one individual react w/ Ags of another individual

Alloimmune hemolytic anemia

Cold agglutinin - Alloimmune vs. autoimmune

Cold autoimmune

Cold agglutinin - CBC results

↓ RBC, Hb, Hct ↑ MCV and RDW

Cold agglutinin - RBC morphology

Clumping of RBCs due to IgM Ab

Ab associated with paroxysmal cold hemoglobinuria

IgM?

2 disorders commonly associated w/ microangiopathic hemolytic anemia (MAHA)

- Hemolytic uremic syndrome (HUS) - Thrombotic thrombocytopenic purpura (TTP) - Disseminated intravascular coagulation (DIC)

Predominant type of poik found in MAHA

Schistocytes

N/N Anemias Flashcards

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