24-year-old male, previously healthy.
She presented with a 7-day history of colic pain in the left lumbar fossa associated with moderate hematuria.
A renal colic was diagnosed, indicating outpatient treatment.
In the following four days, the patient had a torpid course, with worsening of the general condition and severe dyspnea.
Physical examination revealed fever, jaundice and purpuric lesions in the arms.
The clinical picture worsened in the first hours of hospitalization, with generalization of purpura and impaired consciousness that culminated in tonic-clonic seizure, without neurological focalization or signs.
It was decided to enter the Intensive Care Unit (ICU), connecting him to mechanical ventilation (MV).
Multiple samples were taken for bacterial culture and broad-spectrum antibiotic therapy was initiated.
The hemogram showed an erythrocyte count (GR) of 2.59 10°/mm3, hemoglobin (Hb) of 7.3 g/dL, hematocrit (Hto) of 21.4% x corrected platelet count of 10 mm3.
Indirect bilirubin: 3.56 mg/dL, LDH: 2.477 U/L, creatinine (Cr): 1.8 mg/dL and urea nitrogen (NU): 27.8 mg/dL.
Active urinary sediment with hematuria (30 erythrocytes per field) and proteinuria 300 mg/dL in an isolated sample.
Coagulation tests, immunological and microbiological studies were negative.
Myelogram showed marked erythroblastic hyperplasia.
Chest radiography, abdominal ultrasound and brain computed tomography (CAT) showed no relevant findings.
ADAMTS activity was not measured 13.
On the second day of hospitalization, with the diagnosis of TTP, plasmapheresis was started, with an average change of 1.5 daily blood volume (30 ml/kg).
On the sixth day, the patient remained febrile, anemic, with a hemoglobin Hb of 8.1 mg/dL, platelet count of 7 x 103/mm3, LDH of 2,600 U/L, indirect bilirubin of 1.4 mg/dL.
Methylprednisolone (1.5 mg/kg) was added at non-umbilical doses.
Fourteen days later, after a new seizure and without evidence of clinical or laboratory improvement, vincristine was added at a dose of 1 mg iv, repeated 18 days later.
1.
Associated etiology was established with recovery of pathological parameters and renal function.
Epistaxis and purpuric lesions disappeared and seizures did not recur.
On day 18 of hospitalization, plasmapheresis and corticosteroids were suspended.
1.
On the 26th day of hospitalization, asymptomatic and with practically normal laboratory values, it became intermediate.
The patient was discharged 40 days later.
At 18 months of follow-up there were no signs of disease recurrence.
