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1.3.1.4Chdiak-Higashi anomaly
- Characterized by albinism, recurrent infections,
hemorrhagic tendencies, and giant lysosomes in all
granule-containing cells.
- Thrombocytopenia does no occur, the
prolongation of the bleeding time exceeds what
would be expected based on platelet counts.
- The ratio of ATP to ADP in these platelets is
consistent with granule deficiency.
- The platelet response to ADP, epinephrine,
collagen, arachidonic acid, and the calcium
ionophore A231987, although variable usually is
deficient.
- Typically, it is secondary wave of aggregation that
is normal or absent.
1.3.2. Granule-Release Defect
- Clinical picture is the same with storage pool
defect, except that the stored contents of the
and granules are normal.
Conditions associated:
1.3.2.1. Deficiencies of Platelet Prostaglandin
Enzymes
1.3.2.2. Cyclooxigenase Deficiency
1.3.2.1. Deficiencies of Platelet Prostaglandin
Enzymes
- Includes defects in cyclooxygenase and abnormal
thromboxane A2 activity.
- Abnormal platelet aggregation curve obtained by
the addition of arachidonic acid to platelets
deficient in cyclooxygenase is corrected by the
addition of prostaglandin G2.
1.3.2.2. Cyclooxigenase Deficiency
- Patient may present with mild bleeding disorder
- Treatment usually consists of avoiding antiplatelet drugs and controlling menorrhagia with
hormonal therapy.
- Thrombin will cause platelet release by way of a
mechanism other than prostaglandin.
2. Acquired Qualitative platelet disorders
Agents
2.1 Drugs
2.2 Diet
2.3 Diseases
2.1 Drugs
- Aspirin, penicillins, and alcohol are the most
frequently reported cause of clinical bleeding
problems.
- Aspirin and alcohol have a synergistic effect in
increasing the bleeding time.
2.3. Diseases
Conditions involved:
2.3.1. Myeloproliferative Disorders
2.3.2. Uremia
2.3.3. Disseminated Intravascular Coagulation
2.3.4. Immunoglobulin Production