Hemolytic Anemias
Defined as those anemias result from an increased in the rate of red cell destruction.
The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen].
Acute destruction or chronic destruction Extravascular or Intravascular hemolysis Hereditary or Acquired hemolytic anemia
SJAOUNI, KAU
SJAOUNI, KAU
Classification of hemolytic anemiasHereditary Acquired
Membrane
Hereditary spherocytosis, hereditary elliptocytosis
Immune
Autoimmune Warm antibody type
Metabolism Cold antibody type
G6PD deficiency, pyruvate kinase deficiency
Alloimmune
Hemolytic transfusion reactions
Hemoglobin
Thalassemia disorders Hemolytic disease of the newborn Allografts, especially marrow transplantation
Abnormal (Hb S, Hb C, unstable); Drug associated
Red cell fragmentation syndromes
Arterial grafts, cardiac valves
SJAOUNI, KAU
cont. of Classification of hemolytic anemias
Hereditary Acquired
Microangiopathic
Thrombotic thrombocytopenic purpura Hemolytic uraemic syndrome Meningococcal sepsis Pre-eclampsia Disseminated intravascular coagulation
March hemoglobinuria
Infections
Malaria, clostridia
Chemical and physical agents
Especially drugs, industrial/domestic substances, burns
Secondary
Liver and renal disease
Paroxysmal nocturnal hemoglobinuriaG6PD, glucose-6-phosphate dehydrogenase; Hb, hemoglobin.
Diagnosis
History Clinical features Laboratory findings
CBC (Hb & MCV), Plt, (WBC & diff) Retic Blood film Specific tests
SJAOUNI, KAU
CBC
WBC 8.3 10^3/L
NE % 64.5 %
LY % 27.6 %
MO % 5.1 %
EO % 2.6 %
BA % 0.2 %
NRBC %0.0 %
NE # 5.3 10^3/L
LY # 2.3 10^3/L
MO # 0.4 10^3/L
EO # 0.2 10^3/L
BA # 0.0 10^3/L
NRBC #0.0 10^3/L
RBC 4.94 10^6/L
HGB 14.3 g / dL
HCT 40.8 %
MCV 82.6 fL
MCH 28.9 pg
MCHC 35.0 g / dL
RDW 12.8 %
PLT 268 10^3/L
MPV 7.5 fL
RET %
RET #
IRF
MRV SJAOUNI, KAU
Laboratory findings in Hemolytic Anemia1. Features of increased red cell destruction:
S. bilirubin, urine urobilinogen breakdown, fecal stercobilinogen, s. haptoglobins absent.
2. Feature of red cell productions Reticulocytosis BM erythroid hyperplasia
3. Damaged red cell Morphology Osmotic fragility & autohemolysis Red cell survival
SJAOUNI, KAU
Autoimmune Hemolytic Anemia (AIHAs)
Caused by antibody production by the body against its own red cells.
Characterized by a positive direct antiglobulin test (DAT) called Coombs test
Divided into warm and cold according Abs react at 37oC or 4oC.
SJAOUNI, KAU
SJAOUNI, KAU
Autoimmune Hemolytic AnemiaWarm Type Cold Type
Idiopathic Idiopathic
Secondary: Systemic lupus erythematosus, other connective tissue disorders
chronic lymphocytic leukaemia
malignant lymphoma
ulcerative colitis
ovarian teratoma
drugs, e.g. methyldopa
Secondary: Mycoplasma pneumonia
infectious mononucleosis
malignant lymphoma
paroxysmal cold hemoglobinuria: rare; may be primary or associated with infection
Cold Autoimmune Hemolytic AnemiaIn these syndromes the antibodies whether: Monoclonal: Idiopathic cold agglutination
lymphomas. Polyclonal as infection: Infectious
mononucleosis, Mycoplasma pneumonia Patient may have a chronic hemolytic anemia
aggravated by cold. Sometimes associated with intravascular hemolysis.
Laboratory: Similar like warm AIHA Less spherocyte, more red cell agglutination DAT reveals complement (C3d)
SJAOUNI, KAU
SJAOUNI, KAU
Causes of intravascular hemolysis
Mismatched blood transfusion (usually ABO)
G6PD deficiency with oxidant stress
Red cell fragmentation syndromes
Some autoimmune hemolytic anemias
Some drug- and infection-induced hemolytic anemias
Paroxysmal nocturnal hemoglobinuria
March hemoglobinuria
Unstable hemoglobin
G6PD, glucose-6-phosphate dehydrogenase.
Causes of hereditary hemolytic anemia.
SJAOUNI, KAU
Hereditary Hemolytic Anemia
Membrane defects Metabolic defects Hemoglobin defects
hereditary deficiency of: defective synthesis,
spherocytosis e.g. thalassemia
pyruvate kinase ( or )
hereditary
elliptocytosis triose phosphate abnormal variants,
isomerase e.g. Hb S, Hb C,
hereditary unstable
stomatocytosis pyrimidine-5-
nucleotidase
etc. glucose-6-phosphate
dehydrogenase
glutathione synthetase
etc.
INHERITANCE
■ AUTOSOMAL
■ SEX-LINKED
SJAOUNI, KAU
SJAOUNI, KAU
Sex-linked Inheritance
Glucose-6-Phosphate Dehydrogenase (G6PD)Deficiency
Sex-linked hereditary hemolytic anemia Acute intravascular hemolysis due to oxidant stress. Episodes of acute hemolytic anemia
Most patients are asymptomatic 4 Clinical syndromes:
1. oxidative stress-induced hemolysis by certain drugs, infection and other illness.
2. Favism (fresh and uncooked beans are more dangerous than dried cooked ones.
3. Neonatal jaundice4. Chronic hemolytic anemia
SJAOUNI, KAU