ISSN 1866-8836
Клеточная терапия и трансплантация

PC-01. Immune-mediated damage of nervous system after hematopoietic stem cell transplantation

Natalia V. Bronina, Bulat M. Kurmanov, Evgeny A. Burtsev, Georgy Z. Seregin, Maria V. Natrusova, Inna O. Shchederkina, Gleb O. Bronin, Ella V. Kumirova, Michael A. Maschan

Morozov City Children’s Hospital, Moscow, Russia


Contact: Dr. Natalia V. Bronina, phone: +7 (919) 770-22-78, e-mail: Nata-dim@mail.ru

doi 10.18620/ctt-1866-8836-2023-12-3-1-176

Summary

The diagnostics, differential diagnosis and treatment of neurological complications after hematopoietic stem cell transplantation (HSCT) seems to be a complex issue, due to the variety of causes of CNS damage in post-transplant patients, complexity of interpretation, the need to attract additional experts and lack of established therapeutic algorithms with proven effectiveness. Our objective was to study our own experience in diagnostics and treatment of neurological immune complications in children after HSCT.

Materials and methods

During the time period from 01.01.2020 to 30.06.2023, 284 HSCTs were performed at the Morozov Children’s Hospital, Moscow. Of these, five patients (2 boys, 3 girls) were diagnosed with immune-mediated CNS damage (1.7%). Their average age was 8 years (2 to 18 years old). The underlying diseases were: beta-thalassemia, acute myeloid leukemia, myeloid sarcoma, acute lymphoblastic leukemia and juvenile myelomonocytic leukemia. In two patients, neurological complications developed after the 2nd HSCT; in 3 patients, after the first transplant. In most cases (4/5), HSCT was performed from a haploidentical related donor. In one case, a 10/10 matched unrelated donor was used. All patients underwent a comprehensive examination, which included dynamic monitoring of neuroimaging and laboratory diagnostics, including a spectrum of specific antibodies in peripheral blood and cerebrospinal fluid (CSF) as well as electrophysiological studies.

Results

In four children, the appearance of specific complaints was noted before day +100 after HSCT, in 1 child, after day +100 post-transplant. Clinical and laboratory criteria of macrophage activation syndrome associated with HSCT were verified in all patients. All patients underwent blood tests and CSF examination. One child had albumin/cytological dissociation in CSF, the other patients did not show any changes upon the CSF clinical examination. None of the children exhibited hemophagocytosis in CSF. In one child, human herpes virus type 6 was detected in CSF (8.500 gene copies/μL). Antibodies to glutamate decarboxylase (anti-GAD) were detectable in blood of three out of five children. Neuroimaging revealed limbic encephalitis in two cases, myelitis of the cervical spinal cord and neuritis of the left optic nerve was diagnosed in one patient. For treatment of three children, we used only drug therapy (methylprednisolone, high-dose intravenous immunoglobulin, rituximab, cyclophosphamide); in two children, immunosuppression was used in combination with extracorporeal therapy (plasmapheresis, immunoadsorption). At present, three children are fully recovered. One child died with complications of 2nd HSCT not related to CNS damage. In one child, the immune affection of brain was canceled, but the patient died at the Palliative Care Department due to the development of a severe neurological deficit.

Conclusion

The occurrence of neurological symptoms in patients after HSCT requires a comprehensive examination for the timely verification of the immune genesis of CNS damage, thus allowing to choose a more active therapeutic approach and to improve the results of treatment of neurological complications.

Keywords

Hematopoietic stem cell transplantation, autoimmune encephalitis.


Supplement 12-3
09/30/2023

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doi 10.18620/ctt-1866-8836-2023-12-3-1-176

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