TREATMENT EXPERIENCE in MANAGING IMMUNE-MEDIATED

THROMBOTIC THROMBOCYTOPENIC PURPURA (iTTP) from A DISTRICT

HOSPITAL in SARAWAK – A CASE SERIES

W.J. LAUa, K.Y. YONGb

a
Department of Medicine, Bintulu Hospital, Sarawak, Malaysia.
b
Department of Medicine, Miri Hospital, Sarawak, Malaysia.

Introduction: Thrombotic thrombocytopenic purpura (TTP) was a haematological

emergency characterized by a dyad of microangiopathic haemolytic anaemia (MAHA) and

thrombocytopenia as a result of severely deficient ADAMTS13 activity (< 10%). 95% of

cases were immune-mediated (iTTP) due to autoantibodies against ADAMTS13. Prompt

initiation of therapeutic plasma exchange (TPE) and immunosuppressants improved the

mortality rate from 90% to < 20%. Limited access to a tertiary centre, delay in obtaining

ADAMTS13 results, lacking awareness of TTP presentation and availability of TPE remained

a great challenge in district hospitals. Methods: We identified two iTTP patients from the

year 2011-2022 in Bintulu Hospital. Peripheral blood films (PBF) were shown to the visiting

haematologist by sending the picture of PBF as there was no resident haematologist in

Bintulu Hospital. ADAMTS13 activity and inhibitor level were taken prior to TPE initiation.

Results: Patients 1 and 2 were 41 and 44 years old Ibanese gentlemen with good premorbid.

Patient 1 complained of a 3-day history of headache and abnormal behaviour while patient 2

presented with a 1-week history of epigastric pain. Initial blood parameters showed

haemolytic anaemia and thrombocytopenia. (Patient 1: Haemoglobin 7.6g/dL; Platelet

1x109/L; Patient 2: Haemoglobin: 6g/dL; platelet 12x10 9/L). PBF demonstrated presence of

MAHA with > 10% of schistocytes and true thrombocytopenia. Their PLASMIC scores were

high (6 points). In view of strong clinical suspicion of iTTP, both were commenced of TPE

and immunosuppressants immediately after discussing with visiting haematologist via phone.

The diagnosis of iTTP was confirmed a month later in which both the ADAMTS13 activities

came back as 0% with high inhibitors level. They responded well to the treatment and were

well till now with no sign of relapse. Conclusion: We surmised that early detection of iTTP
by astute awareness with the aid of the PLASMIC score in a district setting could immensely

improve the clinical outcome and survival after a timely intervention.