Quiz 3

(Ref: Harrison 20th edition, p 714-15; Harrison 19th edition, p 656)
Clinical features of G6PD
1. G6PD confers a relative resistance against falciparum malaria and is XLR in inheritance.
2. Majority of people with G6PD deficiency remain clinically asymptomatic throughout their lifetime.
3. On the contrary, all of them have an increased risk of developing neonatal jaundice, with peak incidence of clinical onset is between day 2 and day 3.
4. Increased Risk of developing acute hemolytic anemias a result of three types of triggers: (1) fava beans, (2) infections, and (3) drugs

(Ref: Harrison 20th edition, p 517; Harrison 19th edition, p 1793)
• WBC counts are in excess of >100,000/cumm and leads to leukostatic plugging of the capillaries followed by endothelial damage to blood vessels.
• The clinical presentation of leukostasis indicates neurologic and pulmonary pathology.
• CNS leukostasis presents with stupor, headache and dizziness. Administration of 600cGY of whole brain irradiation can protect against this complication along with anti-leukemic therapy.
• The presence of dysnea, tachypnea and respiratory distress indicates pulmonary leukostasis.
• The mortality occurs due to respiratory failure, intracranial haemorrhage and coma.
• The low PO2 is due to increased consumption of oxygen by leucocytes.
• It is common with Acute leukemia subtypes like Acute promyelocytic leukemia, acute monocytic leukemia and T cell type of ALL

(Ref: Harrison 20th edition, p 835-36; Harrison 19th edition, p 737)
Laboratory findings in disseminated intravascular coagulation
1. Prolongation of PT and or APTT
2. Platelet counts < 100,000/cu.mm or a rapid decline in platelet numbers
3. Presence of schistocytes fragmented red cells in the blood smear an
4. Elevated levels of FDP
5. The most sensitive test for DIC is the FDP level
 DIC is an unlikely diagnosis in the presence of normal levels of FDP
 The D dimer test is more specific for detection of fibrin.
 Fibrinogen degradation products indicates that the cross linked fibrin has been digested by plasmin
 Because fibrinogen has a prolonged half-life plasma levels diminish acutely only in severe cases of DIC
 High grade DIG is also associated with levels of antithrombin III or plasminogen activity < 60% of normal

(Ref: Wintrobe's Hematology 12th edition, p 1683)
• Platelet concentrates may be provided by the blood bank in their individual plastic bags or pooled before transfusion. Once the blood bag is opened by puncturing one of the sealed ports, the platelets must be administered within 4 hours.
• Platelets must be administered through a filter approved for platelet use, either a standard 170-lam filter or a leukoreduction platelet filter