Graft-Versus-Host Disease Myopathy

Clinical History:

The patient is a 62-year-old woman with myalgia and symmetric proximal weakness.

PMH:

• Myelodysplastic syndrome (MDS)
• Complicated by acute myelogenous leukemia
  • Karyotype (43,XX,-5,-7,del(17)(p13),+8,+21)
  • Refractory to chemotherapy
  • Status-post stem cell transplant from HLA-matched unrelated donor.

Meds: Long-term steroids

MRI of thigh shows T2 bright signal in fascia

Labs:

• CK 1050 U/L
• Extended myositis associated antibody panel (including HMGCR and cN1a): negative

What stem cell transplant has the lowest rates of this complication?

A. Full HLA-matched sibling

B. HLA-matched unrelated donor

C. HLA-matched cord blood

D. HLA-mismatched unrelated donor

Answer:

HLA-matched sibling donor has the lowest rates of Graft-vs-Host Disease (GVHD)

Graft-versus-Host disease (GVHD)-associated myopathy is a rare complication of allogeneic hematopoietic stem cell transplantation usually occluding in the setting of previously diagnosed chronic GVHD. It presents with subacute or chronic, symmetric, proximal weakness, elevated CK and negative myositis-associated serology. Histologically it may be indistinguishable from overlap myositis; however, it may also involve perimysial and fascia connective tissue and show small vessel/capillary loss.

All allogenic stem cell transplants have some risk of GVDH. HLA-mismatched unrelated donor SCT has the highest risk for GVHD, while full HLA-matched sibling’s have the lowest risk. HLA-matched cord blood is relatively low risk but can suffer from poor engraftment. HLA-matched unrelated donor SCT has intermediate risk.

Reference(s) / Additional Reading:

• Pestronk, A. J Neuropathol Exp Neurol. 2020: 79(1); 102–112.