Incidence of familial Hodgkin's disease

Abstract

Summary The family histories of 131 patients with histologically defined Hodgkin's disease (HD) were studied and 2,517 first and second degree relatives and spouses were identified and followed-up. The causes of death in deceased relatives were ascertained from death certificates. The numbers of deaths from selected causes were compared with the numbers that would be expected if the relatives had suffered the same mortality rates as the Scottish national population. A 4-fold increase in deaths due to HD was found among first and second degree relatives of patients with the disease (6 cases observed compared with 1.4 expected). Five of the 6 familial cases were related to index patients with the mixed cellularity form of the disease, the remaining case was the brother of a patient with the lymphocyte-depleted form of the disease. The increased risk was seen among relatives of both young and older patients and there was no consistent intrafamilial similarity in age of onset or time of onset of disease. (Thompson, 1981) suggests an autosomal recessive HD-susceptibility gene. Case reports of HD occurring in families with known immunodeficiency disorders (McBride & Fenelly, 1977;Buehler et al., 1975;Harris et al., 1981) and the suggested association between HD and certain HLA haplotypes are consistent with the hypothesis that inherited susceptibility to HD may be determined by immune response genes (Marshall et al., 1977;Bowers et al., 1977). Recent epidemiological data suggest that HD, at least in young people, may be a rare sequel to a common virus infection. It has been postulated that a decreased exposure to infections in childhood and a later-than-average age at infection with a common virus may predispose towards the development of the disease (Gutensohn & Cole, 1981).It is also possible that there is more than one aetiology for the disease depending upon histological type (Lukes et al., 1966) onset (Cole et al., 1968;Gutensohn & Cole, 1977). With these possibilities in mind we have investigated the causes of death of first and second degree relatives and spouses of patients with HD. Materials and methods Index casesTwo hundred and three cases of HD were identified in the pathology records of the Dundee hospitals for the 31-year period, 1950-1980 (Lukes et al., 1966).Pedigrees of first and second degree relatives were completed for 131 (71%) of these 170 patients. The histological subtypes and ages at onset are shown in Table I. PedigreesDeceased patients First and second degree relatives and spouses of decreased patients were identified using Scottish Registration records which contain details of all births, marriages, and deaths in Scotland since 1855. Annual indexes are arranged alphabetically by surname for each sex, listing the place of registration and, from 1929 onwards, the mother's maiden name. Sibships were assembled by searching through the birth indexes from the year of the parent's marriage to the year in which the

Familial aggregation of Hodgkin lymphoma and related tumors

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Exome sequencing reveals germline NPAT mutation as a candidate risk factor for Hodgkin lymphoma

“…20 Association to human leukocyte antigen and linkage to non-human leukocyte antigen loci have been suggested in familial HL, and the genetic susceptibility seems mainly to be related to the disease in young adults. 6,7,[21][22][23] The interaction of genes and environmental determinants, such as viral infections, have also been implicated, but, for example, EBV is detected in the tumor cells of less than one-third of familial HL patients compared with 40% to 50% in sporadic cases.…”

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