Graft-Versus-Host Disease

Published on 05/03/2015 by admin

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Last modified 05/03/2015

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Graft-Versus-Host Disease

• Multiorgan disorder that most commonly results from the transfer of donor hematopoietic stem cells into a recipient via an allogeneic hematopoietic stem cell transplant (HSCT).

• Despite advances in HSCT procedures and post-transplantation immunosuppressive therapy, more than half of HSCT recipients develop chronic graft-versus-host disease (GVHD), which remains a major cause of morbidity and mortality.

• Table 44.1 lists risk factors for GVHD in HSCT recipients.

Table 44.1

Risk factors associated with the development of graft-versus-host disease (GVHD).

HLA, human leukocyte antigen.

• Less frequent settings of GVHD include transfusion of non-irradiated blood products to an immunocompromised patient, maternal–fetal transmission to an immunodeficient neonate, and solid organ transplantation.

• Divided into acute and chronic forms based on clinical features.

• Pathogenesis of acute GVHD occurs in three steps: (1) HSCT conditioning regimen leads to epithelial cell injury and activation of host antigen presenting cells; (2) activation of donor T cells; (3) tissue destruction by cytotoxic T cells, natural killer cells, and soluble factors (e.g. tumor necrosis factor-α [TNF-α]).

• Chronic GVHD shares features (e.g. autoantibody production, cutaneous sclerosis) with autoimmune connective tissue diseases.

• Histologically, acute GVHD and epidermal involvement in chronic GVHD are characterized by variable degrees of keratinocyte necrosis (often accentuated in appendages), vacuolar degeneration of the basal layer, and a band-like lymphocytic infiltrate.

Acute GVHD

• Most often arises 4–6 weeks after HSCT with traditional regimens.

• Persistent, recurrent, and late-onset variants of acute GVHD can occur during a time period (>100 days post-transplant) traditionally reserved for chronic GVHD, especially following interventions such as tapering of immunosuppression and donor lymphocyte infusions.

• Typically presents as a morbilliform exanthem, often with perifollicular accentuation.

• Initial predilection for acral sites (e.g. dorsal hands and feet, palms, soles, ears), forearms, and upper trunk.

• Clinical staging is based on the proportion of the cutaneous surface involved: Stage 1, <25%; Stage 2, 25–50%; and Stage 3, >50%. Stage 4 represents erythroderma with bullae/epidermal detachment resembling toxic epidermal necrolysis (Fig. 44.1).

Fig. 44.1 Clinical spectrum of acute cutaneous graft-versus-host disease. A Stage 1 – discrete and coalescing small pink papules on the upper chest and neck of a woman 6 weeks following allogeneic bone marrow transplant. B, C Stage 2 – pink macules and papules of the palms that are becoming confluent 14 weeks post allogeneic bone marrow transplant and pink-violet macules and minimally elevated papules on the abdomen in a liver transplant recipient. D Stage 3 – diffuse erythema with desquamation, but without bullae formation. E Stage 4

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