Acquired defects of the red cells
membrane
Paroxysmal nocturnal haemoglobinuria
(PNH)
Unknown etiology .
Characterized by chronic
intravascular haemolysis with exacerbations associated with haemoglobiniuria
and marked thrombotic tendency
Due to the presence of an
abnormal haemopoietic clone , which is responsible for the production of a
variable proportion of erythrocytes , granulocytes and platelets.
·
The clinical and
haematological features of PNH include
intavascular haemolysis in the early morning .
venous thrombosis , involving unsual sites .
iron deficiency anaemia .
. pancytopenia reduction
in granulocytes and platelets as well as anaemia .
associated with red cell lysis demonstrable in
fresh acidified serum – Ham’s test or in solutions of low ionic strength – the
sucrose lysis or sugarwater ‘test . the most usually screening test is the
demonstration of haemosiderin in the urine.
The mainstay of treatment for PNH is blood
transfusion , oral , iron oral anticoagulation is indicated for patients
showing a thrombotic tendency.
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