ESPN 51th Annual Meeting

ESPN 2018


 
A REFRACTORY CLINICAL CASE OF LUPUS NEPHRITHIS WHO UNDERWENT BONE MARROW TRANSPLANTATION
NESRIN TAS 1 SELCAN DEMIR 2 DENIZ CAGDAS AYVAZ 3 FATIH OZALTIN 1 BARIS KUSKONMAZ 4 ILHAN TEZCAN 3 SEZA OZEN 2 DICLEHAN ORHAN 5 REZAN TOPALOGLU 1

1- HACETTEPE UNIVERSITY FACULTY OF MEDICINE, DIVISION OF PEDIATRIC NEPHROLOGY
2- HACETTEPE UNIVERSITY FACULTY OF MEDICINE, DIVISION OF PEDIATRIC RHEUMATOLOGY
3- HACETTEPE UNIVERSITY FACULTY OF MEDICINE, DIVISION OF PEDIATRIC IMMUNOLOGY
4- HACETTEPE UNIVERSITY FACULTY OF MEDICINE, DIVISION OF PEDIATRIC HEMATOLOGY
5- HACETTEPE UNIVERSITY FACULTY OF MEDICINE, DIVISION OF CLINICAL PATHOLOGY
 
Introduction:

 Systemic lupus erythematosus is a heterogeneous, multisystem disease characterized by highly diverse clinical manifestations. Hematological involvement significantly effects mortality and morbidity and may cause poor prognosis. Bone marrow transplantation is a potential treatment modality for refractory disease. 

 

Material and methods:

We present a 17 year old girl with lupus nephritis, who underwent bone marrow transplantation.

Results:

 The patient presented with malar rash and arthralgia at the age of 12 years. Her sister was also diagnosed SLE and died elsewhere. She was diagnosed with SLE based on clinical findings and positive ANA, antidsDNA, low compleman C3 and C4 levels. Her urine protein was 449 mg/day. Renal biopsy was reported as class 2 lupus nephritis. Peroral corticosteroid and plaquanil treatment was started but as soon as it was tapered, relapse was observed. Proteinuria increased and the second biopsy was performed and reported as class 4 lupus nephritis. Six doses of pulse MPZ and intravenous cyclophosphamide treatments were administered. After 8 months, rituximab therapy was started to the patient with uncontrolled disease. After six doses of rituximab therapy, she was in remission. Two years after the clinical remission whit the maintenance treatment, she presented with a complaint of arthritis, arthralgia and fever. She had thrombocytopenia (55000/uL) and neutropenia (1300/uL). Bone marrow aspiration and biopsy were taken repeatedly. A possible diagnosis of myelofibrosis due to lupus, or aplastic anemia that may result in autoimmunity were predicted. The case was finally diagnosed as bone marrow aplasia. She was genetically investigated for her family history and ─░KAROS gene defect was detected. A successful allogeneic bone marrow transplantation was performed to the patient at the sixth year of her illness.

 

Conclusions:

 Allogeneic BMT resulted in clinical remission in this difficult case and drawing attention that its use as a potential therapy for refractory lupus patients.