Hodgkin disease in a patient with common variable immunodeficiency

Abstract

Extensive extralymphatic Hodgkin disease developed in a young man with common variable immunodeficiency manifested by hypogammaglobulinemia, recurrent sinopulmonary infections, and multiple autoimmune phenomena. Both humoral and cell-mediated immune dysfunction were present prior to treatment. After two cycles of chemotherapy, irreversible shock developed, and death occurred secondary to overwhelming infection in spite of prophylactic gammaglobulin replacement. The unusual features of this patient's case of extralymphatic Hodgkin disease in association with a primary immunodeficiency disorder have not been previously reported.

Hodgkin's disease associated with IgA and IgG subclass deficiency

“…In the literature, cases of HD complicating primary hypogammaglobulinaemia have been reported [21][22][23], and sometimes in relatives of patients with CVH [24]. Among 159 predominantly antibody deficiencies, one case of HD in a woman with CVH is reported [25].…”

“…Usually, immunodeficient patients with cancer suffer significantly higher mortality resulting from a low rate of tumour remission following conventional anti-cancer therapy and higher rate of fatal infectious complications during chemotherapy [4,23]. However, patients with PHIS in whom cancer develops may be candidates for vigorous therapeutic intervention, even if there is an added risk due to the underlying predisposition to infections [29].…”

Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma in a Patient with Common Variable Immunodeficiency Syndrome

“…No case of lymphoma was described in a report of 17 patients with granulomatous disease associated with CVID [6]. However, there has been one report of a patient with granulomatous CVID who subsequently developed extranodal Hodgkin’s disease [21]. …”

Frequency of coincident iron deficiency and beta-thalassaemia trait.

“…,B-thalassaemia is the commonest haemoglobinopathy in India. 2 Seventy two per cent of patients with P-thalassaemia trait but without iron deficiency were anaemic compared with 90.4% of those with f-thalassaemia trait and coincident iron deficiency. This difference was highly significant (p < 0.001).…”