Residency Program - Case of the Month

Answers:

1. Chronic graft-versus-host disease which  is a major cause of morbidity and mortality in allogenic stem cell transplantation survivors. In this condition, donor T-lymphocytes attack host tissues and the cytokine production pattern is mostly TH2 type.^[1,2]  A punch skin biopsy of abdomen in our patient confirmed the diagnosis. The microscopic findings were as follows:

Minimal junctional vacuolar alteration and rare single apoptotic/necrotic keratinocytes along the dermal-epidermal junction and very sparse superficial predominant lymphocytic infiltrates with mostly melanophages in the papillary dermis, and sclerosis of the deep reticular dermis, abutting subcutis, creating a prominent linear demarcation between deep dermis and subcutis ("line sign").

      2.  Photopheresis

      3.  T-lymphocyte deactivation to mitigate chronic GVHD

      4.  II - Apheresis is accepted as second-line therapy. 1B - Strong recommendation, moderate-quality evidence.

      5.  We will continue to provide photopheresis until his chemotherapy regimen/schedule plans are finalized.

References:

1. V Ratanatharathorn, L Ayash, H M Lazarus, J Fu and J P Uberti. Chronic graft-versus-host disease: clinical manifestation and therapy. Journal of Bone Marrow Transplantation. July (2) 2001, Volume 28, Number 2, pp 121-129

2. Hakim F, Mackall CL. The immune system: effector and target of graft-versus-host  disease. In: Ferrara JL, Deeg HJ, Burakoff SJ (eds). Graft-vs-Host Disease. Marcel Dekker: New York, 1997, pp 257-289.