XVIII.

Acquired Nonimmune Anemia of

Increased Destruction
Acquired Extracorpuscular Defects (Fragmentation
Syndromes)
Microangiopathic Hemolytic Anemia
(MAHA)
o Disease of small blood vessels
o Disturbance in microvascular
environment
o Changes in small vessels:

Fibrin deposition (follows

vessel injury)

Severe systemic
hypertension

Vessel abnormalities
o Pathophysiology:

Shear force fragment red

cells (caught by
endothelial projections or
partially penetrated the
endothelial cells of the
vessel wall)

Reduce lumen diameter

fibrin depositions

blood pressure
and projections
o Peripheral blood:

Schistocytes (red cell

fragments)

BM compensation
reticulocytosis (larger than
normal cells);
polychromasia

RPI: >3

Thrombocytosis
consumption coagulopathy
o BM:

Normoblastic hyperplasia

Hyperplasia of
megakaryocyte line
o Coagulation:

Factors I, II, V, VIII -

Disseminated Intravascular Coagulation

o Extensive damage to vessel
endothelium
o Exposure to compounds that
initiate clotting
o Thrombocytopenia ( coagulation
factors)
o Granulocytopenia sign of
marrows inability to compensate
o BM: hypocellular
o LD-1 or LD-3
o No age preference
o Abnormal coagulation tests
Thrombotic Thrombocytopenic Purpura

Young adults
Moschkovitz syndrome
Neurologic signs
Renal disease
Vascular occlusions; platelet
aggregation
o Systemic clot formation
o Inappropriate immune response,
decrease in prostaglandin
formation, and stimulation by oral
contraceptives
o Granulocytosis is common
o Normal coagulation tests
o BM: normoblastic hyperplasia
Hemolytic Uremic Syndrome
o Infants, young children
o Renal failure
o GI disturbances
o Dark-colored urine
o Hepatomegaly
o Burr cells (renal disease)
o Methemoglobin
o BUN and serum creatinine
Abnormal Blood Vessel Structure
o Hemangiomas skin, liver
o Abnormal vascular component
o Malignant hypertension
o Thrombocytopenia
o Abnormal coagulation studies
o LD-1 and LD-2
Mechanical Injury to rbcs
o Stress or March Hemoglobinuria

Transient hemolysis
o
o
o
o
o

Acquired Intracorpuscular Mechanisms of Injury

Paroxysmal Nocturnal Hemoglobinuria
o Insidious onset, arises in BM stem
cells
o Abnormal sensitivity to C in
acidified plasma or LSS
environment
o PNH-I
o PNH-II
o PNH-III
o Occur chiefly at night (blood pH
falls)relative reticulocytosis does
not occur
o Relative lymphocytosis
o BM: normoblastic hyperplasia ->
aplastic anemia
o LAP and AChE
o Laboratory Tests:

Sugar water or sucrose

lysis screening test

Hams acidified-serum test

confirmatory test
o Occurs in absence of antibody