anemia part 2 Flashcards
Self-limiting, but severe even fatal following the administration of
drug that can cause immune hemolytic anemia
Drug-Induced immune hemolytic anemia
Usually occurs in newborns following the transplacental passage
of maternal anti-fetal red cells antibody
Alloimmune Hemolytic anemia
Nnemia due to mechanical extracorpuscular abnormality
Microangiopathic hemolytic anemia (MAHA)
Traumatic cardiac hemolytic anemia
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)
is a group of clinical disorders characterized by RBC fragmentation
in the circulation resulting in intravascular hemolysis
Microangiopathic hemolytic anemia (MAHA)
RBC fragmentation
schistocytes or helmet cell
is rare and potentially fatal characterized by disseminated
thrombotic occlusions of the microcirculation.
Thrombotic thrombocytopenic purpura (TTP)
clot formation
microthrombi
responsible for adhesion and application
von Willebrand factor
severe microangiopathic anemia caused by E. coli serotype 0:157
H7
Hemolytic uremic syndrome (HUS)
Hemolytic uremic syndrome (HUS) causes
renal failure
thrombocytopenia (dec platelets)
mucocutaneous hemorrhage
Characterized by a widespread activation of the hemostatic
system, once it was activated it results into fibrin and thrombi
formation
Disseminated Intravascular coagulation (DIC)
is a very serious complication during pregnancy
characterized by
hemolysis, elevated liver enzyme low platelets count
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
also known as Sports anemia
MARCH HEMOGLOBINURIA
Common in athletes and soldiers
MARCH HEMOGLOBINURIA / sports anemia
laboratory findings for Traumatic cardiac hemolytic anemia/ Macrovascular
hemoglobinuria
presence of schistocytes
inc. reticulocytes
dec. platelets
inc. LDH (Lactate dehydrogenase
laboratory findings for MARCH HEMOGLOBINURIA
o Destruction of RBC/Schistocytes
o Decrease Hct and Hgb
o Macrocytic: Tea colored urine
o Increase in Retics
Lab findings for HUS:
schistocytes, gastroenteritis
Lab findings for Microangiopathic hemolytic anemia (MAHA)
anemia, schistocytes
ANEMIA DUE TO INFECTIONS
Malaria
Bartonellosis
Can cause anemia by the rupture of infected cells at the
end of the asexual cycle
malaria
An uncommon hemolytic disorder
It can the transmitted either from deer, mice to humans
by vector (Ixodes dammini) or blood transfusion
Babesiosis
LABORATORY TEST OF ANEMIA DUE TO INFECTION
CBC
Peripheral smear
Reticulocyte count increases because of lysis
Serological test
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