cGvHD,   aGvHD

Polymyositis-related chronic graft-versus-host disease

In September 2018, findings from reported cases in graft-versus-host-disease (GvHD) associated polymyositis (PM) were published in Clinical Case reports. The authors aimed to review data from two reports of polymyositis-related GvHD in order to better understand this rare complication of allogeneic hematopoietic stem cell transplantations (allo-HSCT).

Case 1
  • 48-year-old woman with acute myeloid leukemia (AML), diagnosis in 2013
  • Complete remission (CR) after two courses of chemotherapy
  • The patient underwent allo-HSCT in February 2014 after reduced intensity conditioning
  • New symptoms after 10 months: limb weakness and mild dyspnea after walking without myalgia, skin rash, or obvious dysphagia
Case 2
  • 31-year-old woman with AML, diagnosis 20 months ago
  • CR was achieved with two courses of chemotherapy
  • Allo-HSCT after fifteen months after reduced intensity conditioning
  • Hospitalization because of limb weakness and myalgia for 5 months
Test results for Patients 1 and 2 (data is given as Patient 1 and 2)
  • CK (U/L): 2118 vs 18
  • Lactate dehydrogenase (U/L): 528 vs 241
  • Aspartate aminotransferase (U/L): 89 vs 192
  • Alanine aminotransferase (U/L): 85 vs 137
  • Myoglobin (ng/mL): 1057.90 vs3
  • CRP (mg/L): 0.2 vs6
  • Antinuclear antibodies: 1:100 vs 1:320
  • Myositis autoantibody: negative in both patients
  • Thyroid glands: negative in both patients
Therapy
  • Patient 1
    • increased dosage of prednisone and cyclosporin A (CsA), but no significant result was seen
    • rituximab (100 mg) plus a low dose of intravenous immunoglobulin (IVIG, 5-10 g) weekly was applied for consecutive 6 weeks
    • the patient’s CK level became normal
  • Patient 2
    • oral prednisone and CsA did not improve the patient’s status
    • tacrolimus (3 mg QD) combined with oral methylprednisolone (40 mg QD) was implemented
    • Patient 2 had been followed up for over 1 year and she is in a good condition receiving tacrolimus at 3 mg with methylprednisolone at 12 mg daily

In summary, these case reports show that patients who underwent allo-HSCT can develop T cell-mediated immunologic muscle disorder which is similar to polymyositis with comparable clinical manifestations. Corticosteroids and immunosuppressants are feasible therapy options.

References
  1. Meng L. et al. Polymyositis as a manifestation of chronic graft-versus-host disease after allo-HSCT. Clin Case Rep.2018 Jul 12;6(9):1723-1726. DOI: 10.1002/ccr3.1709. eCollection 2018 Sep.
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