CASE REPORT:

31-year-old female presented with acute onset severe headache and giddiness. Review of systems was unremarkable. MRI brain with MR venogram was normal. Blood work up revealed hemolytic anaemia (Table 1). Autoimmune haemolytic anaemia was ruled out by Coomb’s test. Peripheral smear had schistocytes (Figure 1). A diagnosis of MAHA-TTP was made; Two cycles of emergency therapeutic plasma exchange (TPE) and steroid pulse therapy were given. After an initial improvement, platelet count and hemoglobin immediately dropped after stopping plasma exchange. So, Rituximab was started.

On further workup, ANA profile done showed Anti Smith and Anti U1 RNP positive with low

Complement levels which were suggestive of SLE (Table 1). Patient, currently, does not have clinical features of SLE except for inflammatory arthritis but has propensity to develop symptoms in the future. She was started on HCQ and steroids. ADAMTS13 activity was decreased. Antiphospholipid syndrome was ruled out. A final diagnosis of evolving SLE presenting as TTP was made. With the above measures, her platelet and haemoglobin improved with a drop in LDH. She was discharged with a tapering dose of steroid and HCQ. Patient has been under follow up for the past 4 months and is doing well with steroids and HCQ. Platelet count and hemoglobin have been stable.

DISCUSSION

• Thrombotic thrombocytopenic purpura (TTP) in patients with SLE is extremely rare. The overall incidence of TTP in SLE patients is unclear and has been reported to be as low as 0.5%. A review includes about 40 cases of TTP related to SLE. 1
• Recent research has revealed a subset of patients with lupus who have a TTP-like syndrome distinct from TTP2
• In our case, patient had non erosive arthritis; But, SLE was undiagnosed during presentation with TTP.
• SLE may present with hemolytic anemia, thrombocytopenia, neurologic deficits, fever, and renal insufficiency but the finding of fragmented RBC’s or schistocytes favours the diagnosis of TTP.3
• A positive Coombs test is not against the diagnosis of TTP in this setting.4 In our patient, Coomb’s test was negative.
• More than three quarters of the cases of thrombotic microangiopathy occurring in SLE were associated with normal or near normal ADAMTS13 activity. This suggests a different pathogenetic mechanism than that which occurs in idiopathic TTP1,5 In our case, ADAMTS13 was as low as idiopathic TTP.
• Early initiation of therapeutic plasma exchange(TPE) in patients with SLE presenting as TTP has favorable outcomes. As is the case in idiopathic TTP. In our patient, TPE had to be supplemented with Rituximab to maintain stable blood counts.
• Patient is under follow up for 5 months and has stable blood counts with low dose steroids and HCQ.

REFERENCES: 

1. Musio F, Bohen EM, Yuan CM, Welch PG. Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum. 1998;28:1–19.
2. Matsuyama T, Kuwana M, Matsumoto M, Isonishi A, Inokuma S, Fujimura Y. Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. Thromb Haemost. 2009;102:371–378.
3. Porta C, Bobbio-Pallavicini E, Centurioni R, Caporali R, Montecucco CM. Thrombotic thrombocytopenic purpura in systemic lupus erythematosus. J Rheumatol. 1993;20:1625–6. 
4. Aleem A, Al-Sugair S. Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Acta Haematol. 2006;115:68–73. 
5. Lansigan F, Isufi I, Tagoe CE. Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13. Rheumatology (Oxford) 2011;50:824–829

Author

Dr Azharuddin M
DNB Resident (General Medicine)
Kauvery Hospital, Chennai

Mentor

Dr. Arshad Raja
Associate Consultant – Haematology and Haemato Oncology
Kauvery Hospital, Chennai