Thrombotic Thrombocytopenic purpura

What is Thrombotic Thrombocytopenic purpura?
Statistics on Thrombotic Thrombocytopenic purpura
Risk Factors for Thrombotic Thrombocytopenic purpura
Progression of Thrombotic Thrombocytopenic purpura
How is Thrombotic Thrombocytopenic purpura Diagnosed?
Prognosis of Thrombotic Thrombocytopenic purpura
How is Thrombotic Thrombocytopenic purpura Treated?
Thrombotic Thrombocytopenic purpura References
Drugs/Products Associated with Thrombotic Thrombocytopenic purpura

What is Thrombotic Thrombocytopenic purpura?

Thrombotic Thrombocytopenic purpura results from widespread hyaline thrombi in small vessels. The pathology not fully understood. Thrombotic Thrombocytopenic purpura is related to a deficiency or autoantibody to the protease enzyme which cleaves von Willebrand factor. The accumulation of very high molecular weight multimers of Von Willebrand Factor and endothelial damage are thought to be the causative factors in this condition.

Statistics on Thrombotic Thrombocytopenic purpura

Young female adults are most commonly affected.

Risk Factors for Thrombotic Thrombocytopenic purpura

Autoantibody to the protease enzyme may be produced as a result of:

Drugs - contraceptive pill, cyclosporin, mitomycin, 5-flurouracil, ticlopidine)

Infection - E coli 0157, Shigella dysenteriae, Streptococcus pneumoniae, coxsackievirus, echovirurs, adenovirus.

Tumours

Pregnancy

Systemic Lupus Erythematosus

HIV

Progression of Thrombotic Thrombocytopenic purpura

Patients typically report an acute or subacute onset of symptoms related to neurologic dysfunction, anaemia, or thrombocytopenia.

Neurologic manifestations include alteration in mental status, seizures,hemiplegia,paresthesias, visual disturbance, and aphasia.

Fatigue may accompany the anemia.

Severe bleeding from thrombocytopenia is unusual, although petechiae are common.

Fever occurs in approximately 50% of patients.

Patients also may notice dark urine from hemoglobinuria.

How is Thrombotic Thrombocytopenic purpura Diagnosed?

Full blood count- (decreased Hb, platelets, increased neutrophils)

Urea and electrolytes - increased urea and creatinine,

Lactic Dehydrogenase - increased (the appearance of lactic dehydrogenase is an indication of cell death)

Urinalysis - haematuria, proteinuria

Prognosis of Thrombotic Thrombocytopenic purpura

Untreated condition - mortality of 90%

With treatment - 10% mortality.

How is Thrombotic Thrombocytopenic purpura Treated?

It is a haematological emergency - expert advice required

Plasma exhange -Prednisolone - 1mg/kg dailyLow dose aspirin (75mg) as the platelet count rises above 50 x 109Monitor disease treatment with LDH and platelet count.

Thrombotic Thrombocytopenic purpura References

Kumar P, Clark M. CLINICAL MEDICINE. WB Saunders 2002 Pg 427-430.
Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001

Drugs/Products Used in the Treatment of This Disease:
- Vincristine Sulfate Injection (DBL) (Vincristine sulfate)
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Article Dates:

Modified: 11/2/2008 Created: 31/8/2003