|Year : 1987 | Volume
| Issue : 2 | Page : 105-7
Thrombotic thrombocytopenic purpura (a case report).
PM Parikh, VS Chile, BC Mehta, PD Anjaria
P M Parikh
|How to cite this article:|
Parikh P M, Chile V S, Mehta B C, Anjaria P D. Thrombotic thrombocytopenic purpura (a case report). J Postgrad Med 1987;33:105-7
|How to cite this URL:|
Parikh P M, Chile V S, Mehta B C, Anjaria P D. Thrombotic thrombocytopenic purpura (a case report). J Postgrad Med [serial online] 1987 [cited 2020 Feb 25 ];33:105-7
Available from: http://www.jpgmonline.com/text.asp?1987/33/2/105/5296
Thrombotic thrombocytopenic purpura (Moschocowitz's syndrome or T.T.P.) is a micro-angiopathy characterised by thrombocytopenic purpura, intravascular hemolysis, fluctuating neurological manifestations and renal failure. it is more common in females and occurs around the age of 40 years. It is a rare disease and is fatal in 50 to 80 per cent of cases. It is thought to be due to increased platelet adhesiveness secondary to reduce prostacyclin production by the vascular endothelium., Very few cases have been reported in the Indian literature., The present paper describes one such rare case admitted under our care at the K.E.M. Hospital.
S.K., a 40 year old female, presented with a history of fever, pain in the abdomen, vomiting and loose motions since 8 days. The fever was intermittent, high grade and the loose motions were watery, large in quantity and occurred 5 to 6 times daily. This was followed by reddish, small, patchy, skin lesions which started three days before admission. Two days before admission, the patient developed diminishing urine output and deteriorating consciousness.
On admission, the pulse was 100/minute and regular; the respiratory rate was 28/minute and B.P. was 130/80 mm Hg in the right upper limb in supine position. There was a petechial and ecchymotic rash all over the body but more prominent on the extremities. She also had puffiness of the face and oedema of the feet. There was no icterus. Examination of the respiratory system revealed crepitations in the right infra-mammary region. Central nervous system examination showed that the patient was unconscious. She responded to painful stimulus with purposeful movements of all four limbs. Planters were bilaterally extensor. The rest of the examination was normal and revealed no focal deficit.
Investigations revealed haemoglobin to be 11 gm% with reticulocyte count of 3%. Platelet count was 56,000/cumm. Bleeding time was 9½ minutes and clotting time was normal. Prothrombin time was 17 seconds (control 14 seconds). Partial thromboplastin time was 42 seconds (control 40 seconds). Factor XIII was present. Clot retraction was poor at the end of 4 and 24 hours. The peripheral smear revealed schistocytes, hypochromia and anisocytes. The platelets were markedly reduced with presence of giant forms. BUN was 86 mg% and serum creatinine 10 mg%. Urine output was low and examination showed urine albumin of 2+ and 60-70 RBCs per high power field. SGOT and SGPT were 830 and 180 international units respectively.
RA test was weakly positive. LE test, ANA and dsDNA were negative. C3 level was 50 mg% (normal value 70-110 mg%). VDRL was non-reactive. Serum fibrinogen levels were 656 mg% (normal values 200-400 mg%). Fibrin degradation products were positive in 1 in 5 dilution. Ultrasound revealed the size of kidneys to be 11.6 and 11.9 cm. Parenchymal thickness was increased with decreased echogenicity. The pelvicalyceal system was normal. Other investigations were not contributory.
The patient was treated with mannitol, dexamethasone, aspirin and supportive measures. She rapidly regained complete consciousness in the ward within 72 hours. No fresh petechiae developed after 2 days of admission. All skin lesions disappeared by 5 days. Over a period of 3 weeks, the patient showed progressive improvement in clinical and biochemical parameters. At the time of discharge, her clinical examination was totally normal. BUN and serum creatinine were 15 mg% and 1.4 mg% respectively. Urine examination and hemogram were normal.
Follow up over a period of two months revealed no clinical or biochemical deterioration or recurrence.
The diagnosis of TTP is based on the presence of hemolysis, thrombocytopenia, neurological involvement and renal failure. The other common clinical features seen in more than 90% of the cases include fever, anemia, petechiae and erythema, all of which were present in this case. The age and sex of this patient matched with those commonly affected by this disease. The biochemical, hematological and immunological investigations were compatible with the diagnosis of TTP. Rapid recovery from the neurological manifestations was also characteristic. The only differential diagnosis to be considered was adult hemolytic uremic syndrome (HUS). Onset with fever and gastroenteritis, no progression into chronic renal failure and elevated fibrin degradation products would favour this diagnosis. However, when HUS occurs in adults, it is in relation to pregnancy or oral contraceptives. The diagnosis of TTP is more likely because of persistence of fever, neurological manifestations dominating the clinical picture and lesser degree of renal involvement.
The precipitating or causative factors implicated include pregnancy, surgery, antibiotics, oral contraceptives and abortions. This patient had a preceding febrile illness with gastroenteritis.
TTP has a worse prognosis than HUS. Among those who survive the acute episode, chronic renal failure develops within 9 months. This patient needs to be carefully followed up to check for its occurrence.
Plasma exchange offers the best mode of therapy for this disease. This probably acts by replacing a prostacyclin-stimulating factor which is missing in patients with TTP. As a result, the increased platelet adhesiveness is reversed., When used with fresh plasma, corticosteroids and anti-platelet agents, patients may show dramatic improvement. Splenectomy is reserved for refractory cases.
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