Interesting case presentation,anemia,TTP.

anemia,THROMBOTIC THROMBOCYTOPENIC PURPURA

1. Interesting case presentation - dr aswin Dnb 3 rd year general medicine

2. A 17 years old female presented to us on 04/07/2024. She was brought with complaints of altered sensorium for 2 days. Attenders also gave history of headache and fever for 5 days. There were no other significant history. Past history suggestive of ?migraine was present. Menstrual history : LMP – 01/06/24 (regular cycles) No previous history of hospitalisations present. Acute viral/bacterial meningoencephalitis

3. On examination , she was drowsy and arousable , not obeying commands. Vitals – bp-110/70 mmHg; hr-102/min ; febrile(100.4 F) ; RR-18/min. General examination : well built and nourished, pallor ++ CVS, RS and ABD– normal CNS: GCS -e4v2m5(11/15); power could not be assessed; tone was normal in all 4 limbs ; DTR were just present ; B/L plantar flexor. No signs of meningism. L/E: multiple petechiae with purpura noted over both lower limbs and trunk.

4. Investigations outside revealed anemia with thrombocytopenia(7.4g/dl and 20,000 cells/ cumm ) Routine investigations sent here revealed severe anemia with thrombocytopenia (6.6g/dl and 20,000cells/ cumm ) So, We asked for a peripheral smear/LDH/ retic. Count / and LFT Called upon our Pathologist and asked specifically.---- Severe Anemia with T’Penia ?

5. In the meantime, CT Brain was done to rule out any bleed, and it revealed mild diffuse cerebral edema. Her coagulation profile and renal function tests came normal. Serum electrolytes and ammonia were normal. Reticulocyte count(12%, corrected retic count -6.6%) and LDH (1161) were elevated; LFT revealed indirect hyperbilirubinemia; DCT was negative. Tropical workup, blood and urine c/s were sent and were eventually negative. Hence diagnosis of TTP was made on the same day and Hematologist opinion was obtained to confirm the same and planned for emergency Plasmapheresis.

6. ADAMTS 13 levels were not sent due to logistic reasons/cost restraints. Plasmic score for TTP – 6 points( high risk group -72% risk of severe ADAMTS 13 deficiency) And the patient was taken up for emergency Plasmapheresis. Date Hemoglobin Plt . count LDH PLEX 04/07 6.6g 20,000 1161 Cycle 1 05/07 6.9g 30,000 981 06/07 7.0g 25,000 1200 Cycle 2 08/07 7.2g 35,000 Cycle 3 10/07 6.5g 40,000 452 Cycle 4 12/07 8.1g 1,50,000 382 13/07 8.9g 1,85,000

7. Her ANA turned out to be moderate positive++(Nuclear compartment) ENA profile, MPO and PR3 ANCA were negative. She was initiated on pulsing Steroids and Weekly once Rituximab. Right now, has completed 3 cycles of Rituximab; Stable; and is on tapering dose of steroids and planned to add second immunosuppressant during the next visit.

8. Thrombotic thrombocytopenic purpura

9. TTP Thrombotic thrombocytopenic microangiopathies are a group of disorders characterized by reduced ADAMTS 13 levels and MAHA defined by thrombocytopenia and Fragmented RBCs along with other evidence of hemolysis. TMA – TTP(Immune or Hereditary); ST- HUS; Drug induced TMA ; Complement mediated TMA. Not all episodes of MAHA are caused by a TMA and not all TMA will present with MAHA and thrombocytopenia.

10. PLASMIC SCORE Plasmic score estimates the likelihood of TTP in patients with suspected TMA. Presumptive diagnosis made if score 5 or more. Intermediate score(5), high risk(6 and more) supports initiation with PLEX and corticosteroids whilst awaiting confirmatory reports.

11. Diagnosis confirmed by ADAMTS 13 activity testing (<10%) Autoantibodies against ADAMTS 13 – Immune TTP. Evaluating the causes – Drugs like Checkpoint inhibitors, vaccines(influenza; rabies), antibiotics(rifampicin, ciprofloxacin, cephalexin), antiplatelet drugs like clopidogrel and ticagrelor, statins. Medical conditions – SLE, Covid 19 infection