Case history

Case history #1

A 50-year-old man with a history of Philadelphia chromosome positive (Ph+) acute lymphoblastic leukaemia (ALL) receives a human leukocyte antigen (HLA)-matched unrelated donor (MUD) haematopoietic cell transplantation (HCT). On day 16 post-transplant, the patient develops fever and diffuse maculopapular rash affecting >50% of his body surface area. By day 23 post-transplant, the patient starts developing nausea, abdominal pain, and profuse diarrhoea. On day 35, the patient begins showing signs of jaundice and hepatomegaly on physical examination.

Case history #2

A 30-year-old woman with a history of severe aplastic anaemia received an HLA-matched sibling donor haematopoietic cell transplant. Her transplant and immediate post-transplant course were unremarkable. Engraftment of donor white blood cells occurred on day +15 and full donor chimerism was achieved by day +30. Tacrolimus taper was initiated on day +56, slowly weaned over 4 months, and subsequently discontinued by day +180 as the patient did not display any signs or symptoms suggestive of acute graft-versus-host disease (GVHD). During a routine follow-up visit 1 month following discontinuation of tacrolimus, the patient presents with new-onset painful mouth sores; hyperpigmented skin lesions; and dry, gritty, and painful eyes. Physical examination is consistent with the patient's symptoms.

Other presentations

There may be delayed time to onset and variable frequency and severity of acute GVHD in a number of clinical scenarios.[2][3][4][5][6][7]

  • Following reduced-intensity or non-myeloablative HCT, time to onset of acute GVHD may be later and slower than conventional myeloablative HCT due to the slower engraftment of donor lymphocytes.

  • Following umbilical cord-blood transplantation, acute GVHD generally occurs less frequently, at a later time point, and with decreased severity relative to unrelated donor HCT.

  • Following donor lymphocyte infusion (DLI), the timing of GVHD may be delayed. DLI is generally performed in the setting of persistent or recurrent malignancy following myeloablative or non-myeloablative HCT, and most often administered without immunosuppressive prophylaxis, to take advantage of the graft-versus-tumour effect. The severity and manifestations of GVHD may differ following DLI, wherein diagnostic and/or distinctive features of acute and chronic GVHD appear together.

Chronic GVHD may develop and present in a number of ways:[8]

  • de novo (which generally has a good prognosis),

  • evolving directly from acute GVHD (progressive type, which has a poor prognosis), or

  • following a period of GVHD resolution (quiescent or interrupted type, which has an intermediate prognosis).

GVHD may also present as idiopathic pneumonia syndrome (IPS, also known as non-infectious lung injury), a major complication of HCT that occurs in approximately 3% to 15% of allogeneic HCT recipients.[9][10] Patients present with signs and symptoms of pneumonia, non-lobar radiographic infiltrates, abnormal pulmonary function, and absence of infectious organs determined by bronchoalveolar lavage.[11] The median time to onset of IPS is 14-90 days post-transplant.[12] The role of GVHD and alloreactive donor T cells in the pathogenesis of IPS remains a topic of debate.

In the elderly, the development of acute GVHD may be severe and associated with significant morbidity and mortality.[13] 

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