Repeated haploidentical allogeneic hematopoietic stem cell transplantation with TCR αβ/CD19 depletion in patient with primary myelofibrosis. Case report

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Abstract

Indications of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with primary myelofibrosis are intermediate-2 and high-risk group of DIPSS (Dynamic International Prognostic Scoring System), beginning of the disease in childhood. The other adverse factors affect engraftment and survival after allo-HSCT, example partialy matched donor. But the result of allo-HSCT from matched related donors and result of allo-HSCT from haploidentical donors are comparable. The method for haploidentical hematopoietic stem cell transplantation is αβT-cell-depletion. This is clinical case of αβT-cell-depleted haploidentical hematopoietic stem cell transplantation in patient with primary myelofibrosis, the diagnosis was established in childhood.

About the authors

Elmira I. Kolgaeva

National Research Center for Hematology

Author for correspondence.
Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-1491-620X

клинический ординатор отд-ния интенсивной высокодозной химиотерапии и трансплантации костного мозга с круглосуточным стационаром

Russian Federation, Moscow

Vera A. Vasilyeva

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0003-0904-7385

канд. мед. наук, врач-гематолог, зав. отд-нием иммунохимиотерапии с дневным стационаром для больных после трансплантации костного мозга

Russian Federation, Moscow

Larisa A. Kuzmina

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0001-6201-6276

канд. мед. наук, зав. отд-нием трансплантации костного мозга

Russian Federation, Moscow

Mikhail Yu. Drokov

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0001-9431-8316

канд. мед. наук, врач-гематолог, рук. сектора по изучению иммунных воздействий и осложнений после трансплантации костного мозга

Russian Federation, Moscow

Mariya V. Dovydenko

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-6082-0110

канд. мед. наук, врач-гематолог отд-ния интенсивной высокодозной химиотерапии и трансплантации костного мозга с круглосуточным стационаром

Russian Federation, Moscow

Zoya V. Konova

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-5569-0155

врач-гематолог отд-ния интенсивной высокодозной химиотерапии и трансплантации костного мозга с круглосуточным стационаром

Russian Federation, Moscow

Dmitry I. Chebotarev

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0003-2146-0818

патологоанатом патологоанатомического отд-ния

Russian Federation, Moscow

Alla M. Kovrigina

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-8422-0689

д-р биол. наук, зав. патологоанатомическим отд-нием

Russian Federation, Moscow

Denis V. Kamelskih

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-1118-6969

врач-трансфузиолог отд-ния забора гемопоэтических стволовых клеток, обработки и хранения костного мозга и/или гемопоэтических стволовых клеток

Russian Federation, Moscow

Tatyana V. Gaponova

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-9684-5045

канд. мед. наук, врач-гематолог, зам. ген. дир. по трансфузиологии, зав. отд. процессинга клеток крови и криоконсервирования

Russian Federation, Moscow

Manana A. Sokolova

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0003-1682-7005

канд. мед. наук, врач-гематолог, ст. науч. сотр. консультативного гематологического отд-ния с дневным стационаром по проведению интенсивной высокодозной химиотерапии

Russian Federation, Moscow

Irina N. Subortseva

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0001-9045-8653

канд. мед. наук, врач-онколог, ст. науч. сотр. научно-клинического отд-ния стандартизации методов лечения

Russian Federation, Moscow

Anait L. Melikyan

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0002-2119-3775

д-р мед. наук, врач-гематолог, зав. научно-клиническим отд-нием стандартизации методов лечения

Russian Federation, Moscow

Mikhail A. Maschan

Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0003-1735-0093

д-р мед. наук, врач-гематолог, зам. ген. дир. – дир. Высшей школы молекулярной и экспериментальной медицины

Russian Federation, Moscow

Elena N. Parovichnikova

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0001-6177-3566

д-р мед. наук, зав. отд-нием химиотерапии гемобластозов, депрессий кроветворения и ТКМ

Russian Federation, Moscow

Valerii G. Savchenko

National Research Center for Hematology

Email: kolgaeva@inbox.ru
ORCID iD: 0000-0001-8188-5557

акад. РАН, д-р мед. наук, проф., ген. дир.

Russian Federation, Moscow

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2. Fig. 1. Bone marrow trephine biopsy, 2014 (initial examination). The bone marrow is hypercellular owing to the expansion of granulocytic lineage. There is a marked proliferation of atypical megakaryocytes of normal size which form loose and dense clusters. Erythroid lineage is in moderate amount. Hematoxylin-eosin staining. Magnification 100.

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3. Fig. 2. Bone marrow trephine biopsy, 2014 (initial examination). The degree of fibrosis of the stroma MF-1. Gomori staining. Magnification 200.

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4. Fig. 3. Spleen surgical material. The red pulp is hypocellular, there is a marked thickening and fibrosis of the splenic cords. Morphological picture of the fibrocongestive spleen. Hematoxylin-eosin staining. Magnification 200.

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5. Fig. 4. Bone marrow trephine biopsy, 2020 (one year after repeated allo-HSCT). The bone marrow is hypocellular (relatively to the age norm). The granulocytic lineage is reduced. Megakaryocytes are few in number, small in size with hypolobular hyperchromic nuclei. The erythroid lineage is moderately reduced. Morphological picture of remission of myeloproliferative disease (according to ELN 2013 criteria). Hematoxylin-eosin staining. Magnification 100.

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6. Fig. 5. Bone marrow trephine biopsy, 2020 (one year after repeated allo-HSCT). The degree of stromal fibrosis is MF-0. Gomori staining. Magnification 200.

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