Korean J Hematol.  2005 Jun;40(2):106-110. 10.5045/kjh.2005.40.2.106.

A Case of Systemic Sclerosis Developed after Autologous Hematopoietic Stem Cell Transplantation in the Patient with Multiple Sclerosis

• Kim WS
• Min CK
• Yang DW
• Park YK
• Choi SO
• Kim JS
• Lee H
• Cho CS

• KMID: 2252345
• DOI: http://doi.org/10.5045/kjh.2005.40.2.106

Abstract

Herein, the case of a 43-year-old woman, with a relapsing-remitting variant of multiple sclerosis (MS), which began when she was 34 years of age, and gave rise to severe neurological complications, including progressive paralysis in both legs and visual deterioration, is reported. Despite heavy immunomodulatory treatment, her condition relapsed and became aggravated. At this point, the decision was made to perform autologous hematopoietic stem cell transplantation (HSCT). The enrichment of CD34+ cells was followed by depletion of the peripheral T cells. The post-transplantation course was uneventful, and autoimmune thrombocytopenia developed within 7 months of the HSCT. The patient was treated with cyclosporine (CsA) and oral prednisolone, but subsequently developed systemic sclerosis (SSc). The administration of CsA following the syngeneic/autologous HSCT caused a T lymphocyte-dependent autoimmune disease, which resembled graft-versus-host disease (GVHD). It is quite probable the auto-reactive lymphocytes, which were paradoxically elicited by the CsA during the reconstitution of the immune system, partly contributed to the occurrence of the other autoimmune disease, SSc. To our knowledge, this is the first description of a MS patient, having undergone CD34+-selected autologous HSCT followed by the administration of CsA, who subsequently developed SSc.

Keyword

Multiple sclerosis; Systemic sclerosis; Autologous GVHD; Hematopoietic stem cell transplantation; Cyclosporine

MeSH Terms

Adult
Autoimmune Diseases
Cyclosporine
Female
Graft vs Host Disease
Hematopoietic Stem Cell Transplantation*
Hematopoietic Stem Cells*
Humans
Immune System
Leg
Lymphocytes
Multiple Sclerosis*
Paralysis
Prednisolone
Purpura, Thrombocytopenic, Idiopathic
Scleroderma, Systemic*
T-Lymphocytes
Cyclosporine
Prednisolone

Figure

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  Fig. 1. Brain magnetic resonance image (MRI): An axial T2-weighted image shows abnormal non-heme iron deposition in the basal ganglia and thalami.

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  Fig. 2. (A) The hands show tightening of the skin. (B) Ulcers appear on the fingertips.

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  Fig. 3. C omputed tomography of the chest reveals ground-glass opacities in both lower lobes at prone position, suggesting fibrosis.

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