Aplastic anemias &Secondary Anemias Flashcards by Shadekiel Kassie

What is Aplastic Anemia

Defined as pancytopenia resulting from aplasia of the bone marrow

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Describe the marrow in Aplastic Anemia

Hypo cellular Marrow in which normal haemopoietic marrow replaced by fat cells

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What is the pathophysiology of Aplastic Anemia

A reduction in the number of hematopoietic pluripotential stem cells

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What is pancytopenia

Describes a reduction in the blood count of all major cell lines- red cells, white cells and platelets

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Aplastic Anemia is classified into which two types

Primary (congenital or acquired)

Secondary

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What are the two types of primary causes of Aplastic Anemia

Congenital

Idiopathic acquired

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What are the two types of Congenital Aplastic Anemia

Fanconi and Non Fanconi Types

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What are the four main secondary causes of Aplastic Anemia

Ionizing radiation
Chemicals
Drugs
Viruses

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List examples of exposure to Ionizing Radiation that may cause Aplastic Anemia

Radiotherapy
Radioactive isotopes
Nuclear power stations

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Exposure to which drugs/ chemicals may lead to Aplastic Anemia

Cytotoxic drugs:
Benzene
Organophosphates
DDT and other pesticides

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True or False

Exposure to Cytotoxic drugs that causes Aplastic Anemia is usually reversible

True

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Which drugs occasionally cause bone marrow aplasia

Chloramphenicol
NSAID’s
Insectisdes
Gold

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List four drugs that regulary cause marrow depression

Busulfan
Cyclophosphamide
Anthracyclines
Nitrosoureas

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List four viruses that cause Aplastic Anemia

Hepatitis non A non B
Parvovirus B19
EBV (rare)
HIV

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Hepatitis non A non B can cause Aplastic Anemia about how many weeks after infection?

6-12 weeks

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Parvovirus leads to transient aplasia of which blood line

Red cell

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What fraction of Aplastic Anemia cases are no underlying cause found

2/3

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What is the pattern of inheritance of Fanconi caused Aplastic Anemia

Autosomal recessive

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When is the onset of Aplastic Anemia

Any age

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When is the peak incidence for aplastic anemia

30 yrs

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Which gender is slightly more susceptible to Aplastic Anemia

Male

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List some clinical features of Aplastic Anemia

Non specific, the symptoms are due to the pancytopenia effect

infections
bruising/ bleeding gums, epistaxis(nose bleed), menorrhagia

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True or false

Lymph nodes, liver and spleen are enlarged in Aplastic Anemia

False

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Describe the colour and size of RBCs in Aplastic Anemia

Normochromic

Normocytic or
Macrocytic (MCV 95-110fl)

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Is the reticulocyte count high or low in Aplastic Anemia

Low

The range in fall of granulocytes in Aplastic Anemia is usually below what value

1.5 x 10^9 / L :

True or false Neutrophil appears normal in Aplastic Anemia

True

In severe cases of Thrombocytopenia in Aplastic Anemia the values fall below what number

20 x 10^9 / L

Does Aplastic Anemia present with abnormal cells in peripheral blood

Nope!

In Aplastic Anemia the bone marrow shows hypoplasia, with loss of haemopoietic tissue which is replaced with what

Fat cells

What is the importance of the Trephine Biopsy

May show patchy cellular areas in a hypocellular background

What is the type of biopsy done to examine bone marrow and test for Aplastic Anemia

Trephine Biopsy

In the trephine biopsy of a pt with Aplastic Anemia, which cells are mainly present

Lymphocytes | Plasma cells

In the trephine biopsy of a pt with Aplastic Anemia, which cells are severely reduced or absent

Megakaryocytes

Severe cases of Aplastic Anemia show what percentage of Marrow cellularity

<25%

What is the peripheral blood criteria for Severe Aplastic Anemia

Granulocytes <0.5 x 10^9 Platelets < 20 x 10^9/l Corrected retic count <1%

What is the criteria for Severe Aplastic Anemia based on Bone marrow trephine

Marked hypocellularity <25% | Moderate hypocellularity 25% -50%

List five tests used to help diagnose aplastic anemia

``` Liver function test to rule out hepatitis Urine hemosiderin Hams test Human leukocyte antigen Cytogenetics studies ```

Why is a liver function test done when diagnosing Aplastic anemia

To rule out hepatitis as the cause

Why is urine hemosiderin test done when diagnosing a plastic anemia

To rule out chronic intravascular hemolysis as the cause

What is Ham’s test

This is a blood test used to diagnose paroxysmal nocturnal hemoglobinuria PNH

What is the name of the blood test used to diagnose paroxysmal nocturnal hemoglobinuria

Ham’s test

Why is Ham’s test done when diagnosing aplastic anemia

To rule out paroxysmal nocturnal hemoglobinuria

What is the name of the blood test used to identify the best donor for bone marrow transplant

Human leukocyte antigen (HLA) typing

Why is human leukocyte antigen typing done when diagnosing aplastic anemia

To determine the best donor for a bone marrow transplant

List three examples of supportive management of patients with a plastic anemia

Packed red blood cell transfusion (ideally WBC depleted) Platelet transfusion Prevention/management of infections

List five examples of specific management for a plastic anemia

Stem cell transplant Immunosuppressive therapy Anabolic steroids High dose methylprednisolone (immunosuppressive agent) Granulocyte macrophage colony stimulating factor GM- CSF

Define stem cell transplant

It is the process by which normal haematopoiesis is establish by infusion of pluripotent stem cells to reestablish marrow function in a patient with defective or damaged bone marrow

List five types of stem cell transplant

``` Syngeneic Allogeneic related match Allogeneic unrelated match Partially mismatched related Autologous ```

Who is a Syngeneic blood donor

Identical twin

Who is an autologous blood donor

Self

What are three sources of stem cells

Peripheral blood stem cells PBSC Bone marrow Cord blood

List seven early complications of stem cell transplant

``` Nausea and vomiting Mucositis Diarrhea Aplasia Hemorrhagic cystitis Alopecia Hepatic Veno-occlusive disease ```

List four late complications of stem cell transplant

Stability Growth disturbances Cataracts Second-degree malignancies

List three complications of stem cell transplant

Graft rejection Graft versus host disease Leukemic relapse

List seven differential diagnosis of pancytopenia

``` Aplastic anemia Megaloblastic anemia Bone marrow infiltration Myelofibrosis Hypersplenism Proximal nocturnal hemoglobinuria Myelodysplastic syndrome ```

Define Myelofibrosis

It is a rare type of blood cancer in which the bone marrow is replaced by fibrous scar tissue

Define pure red blood cell aplasia PRCA

It is a rare disorder of blood production in which the bone marrow fails to function in an adequate manner resulting in anemia

List one congenital cause of pure red blood cell aplasia PRCA

Diamond Blackfan Anemia

List four cause of Acquired Pure Red Cell Aplasia PRCA

Infection - parvovirus B 19 Thymoma Hematological malignancies Idiopathic

What is another name for a secondary anemia

Anemia of chronic disorder

List three causes of secondary anemia

Chronic infections Inflammatory disorders Malignancy

List five characteristic features of Pure Red Cell Aplasia PRCA Interms of : colour, reticulocyte level, bone marrow invasion, severity

Normochromic normocytic or mildly hypochromic indices Reticulocytes normal or decreased mild non-progressive anemia Does not require bone marrow invasion Severity depends on the disease activity

What is the morphology of the red blood cells in PRCA

Normochromic Normocytic Mildly hypochromic

What is the level of reticulocytes in PRCA

Normal or slightly decreased

When does PRCA usually develop

In the first few months of illness

What is the pathogenesis of PRCA

``` Cytokine mediated process Shortened RBC survival Impaired marrow response Abnormal iron metabolism TNF, IL-1 interferons ```

What is the treatment for PRCA

Treat the underlying cause | Erythropoietin can be used in some cases

What percentage of erythropoietin is produced in the renal cortex

90%

90% of erythropoietin is produced where

Renal cortex

What percent of erythropoeitin is produced in the liver

10%

10% of erythropoietin is produced in which organ

The liver

What is the stimulus for erythropoietin production in the kidney

Oxygen tension

What is the role of Erythropoietin

Increases the number of progenitor cells committed to erythropoiesis

What are the normal blood levels of erythropoietin

11-48 mU/mL ( milliunits per millilitre)

List seven indications for treatment of PRCA with Erythropoietin

``` Chronic renal failure Multiple myeloma Myelodysplastic syndrome Cancer chemotherapy Chronic diseases HIV AZT antiretroviral drug treatment ```

What is the dose of Erythropoietin administered per week To treat PRCA

150 units/kilograms three times per week

The maximum effect of PRCA with erythropoietin occurs in which week of treatment

4-8 week

What is the half time of erythropoietin

4 to 13 hours

Where is erythropoietin metabolized

By the liver

List five effects of treatment with erythropoietin

``` Depletion of iron stores Hypertension, edema Congestive cardiac failure Thrombosis, myocardial infarction, stroke Seizures, headaches ```

Aplastic anemias &Secondary Anemias Flashcards

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