Mycosis Fungoides in Childhood: Description and Study of Two Siblings

Abstract

Primary cutaneous T-cell lymphomas are exceedingly rare in children and adolescents. However, mycosis fungoides (MF) is the most frequent primary cutaneous lymphoma diagnosed in childhood. Two cases of MF in siblings (a 14-year-old boy and his 10-year-old sister) are reported. On the basis of clinical features (histopathological and immunophenotypical findings) a diagnosis of MF patch lesions was made in both siblings. Since recent data in the literature have underlined a high frequency of the HLA-DQB1*03 allele in patients with familial MF (including child patients), the HLA profile of the patients was analysed, indicating the presence of a haplotype (HLA-DQB1*03,*03 in the girl, HLA-DQB1*02,*03 in the boy) corresponding with that described in recent literature. Two rare and exceptional cases of MF in siblings are reported, highlighting the presence of a peculiar haplotype.

HLA Type Determination in Patients Diagnosed with Mycosis Fungoides and Sézary Syndrome

“…Familial aggregation and ethnical differences in incidence point to genetic factors in the susceptibility to MF/SS. 11,12 To the best of our knowledge, scientific literature lacks data about HLA polymorphisms in Turkish MF patients. In this study, we found that HLA-A31, HLA-B51 and HLA-DR3…”

Intravascular large B-cell lymphoma associated with silicone breast implant, HLA-DRB1*11:01, and HLA-DQB1*03:01 manifesting as macrophage activation syndrome and with severe neurological symptoms: a case report

“…There are a few studies that show a higher frequency of HLA-DRB1*11 and/or HLA-DQB1*03 in patients with hematologic malignancies [ 83 ], non-Hodgkin’s lymphoma, diffuse large B-cell lymphoma [ 84 ], mycosis fungoides (cutaneous T-cell non-Hodgkin’s lymphoma) [ 85 – 88 ], acute lymphoblastic leukemia [ 89 ], chronic lymphocytic leukemia [ 90 ], hairy cell leukemia (B-cell leukemia) [ 91 ], and HCV virus-associated lymphoma [ 92 ] compared to controls. These data indicate a probable genetic basis of the lymphomagenesis.…”

“…Our patient’s uncle died at 43 years of age from acute lymphoblastic leukemia. The occurrence of more cases of hematologic malignancies in a family further suggest a genetic contribution to the pathogenesis [ 87 , 88 ].…”

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

“…Purpuric variants, including a case of MF mimicking a pigmented purpuric dermatitis, have also been reported [34,35]. The occurrence of MF in family members (parent-child or siblings) has also been described; a disease susceptibility secondary to specific human leukocyte antigen (HLA) alleles has been proposed to explain this phenomenon [36][37][38][39]. In these instances, the age of onset is often in earlier adulthood than in sporadic cases, typically presenting in the fourth and fifth decade of life, though differences in clinical behavior have not been identified [37,38].…”

“…In these instances, the age of onset is often in earlier adulthood than in sporadic cases, typically presenting in the fourth and fifth decade of life, though differences in clinical behavior have not been identified [37,38]. However, it is notable that "familial MF" has also been reported in siblings during childhood, or affecting both adult parents and their young children, though this occurrence is quite rare [36,37,39].…”