anemia part 2 Flashcards by Ruth Joy De Guzman

Self-limiting, but severe even fatal following the administration of
drug that can cause immune hemolytic anemia

Drug-Induced immune hemolytic anemia

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Usually occurs in newborns following the transplacental passage
of maternal anti-fetal red cells antibody

Alloimmune Hemolytic anemia

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Nnemia due to mechanical extracorpuscular abnormality

Microangiopathic hemolytic anemia (MAHA)

Traumatic cardiac hemolytic anemia

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4 Disorders of MAHA:

Thrombotic thrombocytopenic purpura (TTP)

Hemolytic uremic syndrome (HUS)

Disseminated Intravascular coagulation (DIC)

hemolysis, elevated liver enzyme levels, and low platelet levels (HELLP)

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is a group of clinical disorders characterized by RBC fragmentation
in the circulation resulting in intravascular hemolysis

Microangiopathic hemolytic anemia (MAHA)

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RBC fragmentation

schistocytes or helmet cell

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is rare and potentially fatal characterized by disseminated
thrombotic occlusions of the microcirculation.

Thrombotic thrombocytopenic purpura (TTP)

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clot formation

microthrombi

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responsible for adhesion and application

von Willebrand factor

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severe microangiopathic anemia caused by E. coli serotype 0:157
H7

Hemolytic uremic syndrome (HUS)

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Hemolytic uremic syndrome (HUS) causes

renal failure
thrombocytopenia (dec platelets)
mucocutaneous hemorrhage

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Characterized by a widespread activation of the hemostatic
system, once it was activated it results into fibrin and thrombi
formation

Disseminated Intravascular coagulation (DIC)

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is a very serious complication during pregnancy
characterized by

hemolysis, elevated liver enzyme low platelets count

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anemia is caused by injury and fragmentation RBCs exposed to
high shear stresses on a foreign surface during cardiac surgery.

Traumatic cardiac hemolytic anemia/ Macrovascular
hemoglobinuria

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also known as Sports anemia

MARCH HEMOGLOBINURIA

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Common in athletes and soldiers

MARCH HEMOGLOBINURIA / sports anemia

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laboratory findings for Traumatic cardiac hemolytic anemia/ Macrovascular
hemoglobinuria

presence of schistocytes
inc. reticulocytes
dec. platelets
inc. LDH (Lactate dehydrogenase

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laboratory findings for MARCH HEMOGLOBINURIA

o Destruction of RBC/Schistocytes
o Decrease Hct and Hgb
o Macrocytic: Tea colored urine
o Increase in Retics

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Lab findings for HUS:

schistocytes, gastroenteritis

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Lab findings for Microangiopathic hemolytic anemia (MAHA)

anemia, schistocytes

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ANEMIA DUE TO INFECTIONS

Malaria
Bartonellosis

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Can cause anemia by the rupture of infected cells at the
end of the asexual cycle

malaria

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An uncommon hemolytic disorder
It can the transmitted either from deer, mice to humans
by vector (Ixodes dammini) or blood transfusion

Babesiosis

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LABORATORY TEST OF ANEMIA DUE TO INFECTION

CBC
Peripheral smear
Reticulocyte count increases because of lysis
Serological test

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ANEMIA DUE TO CHEMICAL AND PHYSIAL AGENTS

Drugs and chemicals Venom Thermal injury

Caused by oxidative denaturation of hemoglobin leading to the formation of

i. Methemoglobin ii. Sulfhemoglobin iii. Heinz bodies

example of Drugs and chemicals

i. Naphthalene (Moth balls) ii. Dapsone (for leprosy) iii. Arsenic, copper, lead (for intravascular hemolysis)

Decreased Retics

Anemia due to chronic renal failure  Due to endocrine disorder  Due to marrow infiltration (myelopthisic anemia)  Due to aplastic anemia

Increase Retics

Membrane defect Enzyme deficiency Globin abnormality

damage to RBCs circulating in the involved skin and tissues leading to hemolysis

Thermal Injury

ANEMIA DUE TO MEMBRANE DEFECT

Hereditary Spherocytosis Hereditary elliptocytosis Hereditary stomatocytosis Hereditary pyropoikilocytosis Hereditary acanthocytosis Hereditary RH null disease

Type of inheritance: Hereditary Spherocytosis

Abnormal Autosomal

Type of inheritance: Hereditary elliptocytosis

Autosomal dominant

Type of inheritance: Hereditary pyropoikilocytosis

Autosomal recessive

Type of inheritance: Hereditary stomatocytosis

Autosomal recessive

Also known as Marchiafava-micheli syndrome

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

are chronic defect in RBC membrane considered to be sleeprelated hemoglobinuria

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

yellowish brown crystal that can be found in urine

Hemosidinuria

Special tests for Paroxysmal nocturnal hemoglobin

Ham’s Test/Ham’s Acidified Serum Test Sugar Water Test/Sucrose Hemolysis Crosby’s thrombin test Cobra-venom test Heat Resistance Test Insulin Test

patient’s RBC are exposed to 37 degrees Celsius to the action of the normal or the patient’s own serum suitably acidified to the optimum pH or lysis (pH - 6.5-7.0)

Ham’s Test/Ham’s Acidified Serum Test

patient’s washed red cells are mixed with ABO compatible normal serum and isotonic sucrose

Sugar Water Test/Sucrose Hemolysis

most commonly used tes for PNH

The patient’s red cells are exposed at 37 degrees Celsius to the action of normal own serum which has been suitably acidified and which has been added with commercial preparation of thrombin for lysis

Crosby’s thrombin test

clotted blood is incubated at 37°C and the expected for spontaneous lysis

Heat Resistance Test

most common metabolic disorder of RBCs involving the HMP (aerobic glycolysis) involving hexose monophosphate

G6PD Deficiency

fragmentation occurs as a result of:

RBCs passing through fibrin deposits inside the lumen of arterioles and capillaries  damaged epithelium and vessel walls

most common metabolic disorder of RBCs involving the EMP

Pyruvate Kinase Deficiency

decreased erythrocytes deformability that reduces the lifespan of RBC

Pyruvate Kinase Deficiency

Laboratory findings: Pyruvate Kinase Deficiency

Fluorescent Spot Test (+) Quantitative assay of PK (dec.) ↑ Reticulocyte count

inherited defect that results in abnormal structure of one of the globin chains of the hemoglobin molecule caused by genetic mutations.

Hemoglobinopathy - Globin abnormality

hereditary hemolytic anemia occurs almost always in the Black (Negros) race (Africa and US) the abnormality is due to substitution of valine for glutamic acid in position 6 in the beta chain

sickle cell anemia

sickle cell anemia also known as

drepanocytosis

Laboratory Findings: sickle cell anemia

peripheral smear Decreased OFT Increased Mechanical Fragility Test Positive for Hb Solubility Screening Test Electrophoresis

a benign condition that generally does not affect mortality and morbidity.

sickle cell trait

heterozygous hemoglobin AS state

Sickle Cell Trait

the abnormality is due to substitution of glutamic acid to lysine in the sixth position of the beta chain.

. Hemoglobin C Disease

the abnormality is due to substitution of glutamic acid to lysine in the 26th position of the beta chain

Hemoglobin E Disease

the most common double heterozygous syndrome

Hemoglobin SC

results in a structural defect wherein different amino acid substitutions are found on each of two beta globin chain in the 6th position

Hemoglobin SC

caused by inadequate production of erythropoietin by the kidneys

Anemia due to chronic renal failure

this is the type of anemia associated with diseases of the endocrine glands like hypothyroidism, pituitary deficiency and adrenal gland deficiency.

Anemia due to Endocrine Disorder

Infiltration of abnormal cells into the bone marrow and subsequent destruction and replacement of the normal hematopoietic cells

Anemia due to Marrow Infiltration (Myelopthisic anemia)

is a rare but potentially fatal bone marrow failure that can be acquired or inherited

Aplastic anemia

2 Types of Aplastic Anemia

Acquired Aplastic Anemia Inherited Aplastic Anemia

usually present at an early age and may have associated congenital malformations

Inherited Aplastic Anemia

two types of Inherited Aplastic Anemia

Fanconi Anemia Dyskeratosis Congenita

is an autosomal recessive chromosome instability disorder

Fanconi Anemia

is a rare, inherited bone marrow failure syndrome

 Dyskeratosis Congenita

is an increased concentration of erythrocytes in the blood that is above the normal for age and sex

POLYCYTHEMIA

refers to increase in hematocrit or RBC count due to decreased plasma volume

Relative Polycythemia/ Pseudopolycythemia

Causes of Relative Polycythemia

Acute dehydration Stress

refers to increase in the total red cell mass in the body

Absolute Polycythemia

2 Classifications of Polycythemia

Relative Polycythemia/ Pseudopolycythemia Absolute Polycythemia

Polycythemia Vera other name

True Polycythemia  Erythremia  Vaquez-Osler’s disease  Polycythemia rubra vera

is the special name given to the polycythemia found in association with Congenital Heart Disease

ERYTHROCYTOSIS

2 diseases of ERYTHROCYTOSIS

Congenital Heart Disease (blue babies) Chronic Lung Disease (emphysema)

iron overload and accumulates in the parenchymal cells and injures the tissues

HEMOCHROMATOSIS