"1992 Raven Press, Ltd., New York
J CIin Gastroenterol 1992;15(2):128-35.
Malignant Lymphoma of Jejunum with Common Variable Hypogammaglobulinemia and Diffuse Nodular Hyperplasia of the Small Intestine A Case Study and Literature Review G. Castellano, Ph.D., D. Moreno, Ph.D., 0. Galvao, M.D., c. Ballestin, M.D., F. Colina, Ph.D., M. Mollejo, M.D., J. D. Morillas, M.D., and J. A. Solis Herruzo, Ph.D.
We report a patient with common variable hypogammaglobulinemia and diffuse nodular lymphoid hyperplasia of the small intestine complicated by a jejunal malignant lymphoma. Immunopathological and histological studies showed a polymorphous centroblastic lymphoma with intracytoplasmaticIgM immunoglobulin and lambda light chains. Some mucosal nodules separate from the tumor mass showed atypical lymphoid cell populations similar to lymphoma cells, suggesting a transition between hyperplastic nodules and lymphoma nodules. Four similar cases, and six other patients with malignant lymphoma of the small intestine, associated with diffuse nodular lymphoid hyperplasia, but without immunodeficiency, have already been described. All these cases suggest that nodular lymphoid hyperplasia increases the risk of small intestine lymphoma. Key Words: Common variable hypogammaglobulinemia-Nodular lymphoid hyperplasia-Primary gut lymphomas.
The etiology of the small intestine primary lymphoma remains unknown and probably depends on several causes. An increased incidence of small intestine lymphoma has been found in celiac sprue (l),dermatitis herpetiformis (2), alpha-heavy chain disease (3), regional enteritis (4),and immune system dysfunction of different etiologies (congenital, autoimmune, postransplantation, and AIDS) (5-7). However, most small intestine lymphomas are not associated with any,of these problems (8)A high frequency of malignant epithelial and lymphoid tumors has been noted in common variable hypogammaglobulinemia (CVH) (9-1 1). Nevertheless, to the best of our knowledge, only four small intestinal lymphomas have been described (12-15), all associated with diffuse nodular lymphoid hyperplasia (DNLH). W e report here a new case of small intestine lymphoma in CVH with DNLH.
CASE REPORT A 39-year-old man had frequent bouts of diarrhea and recurrent infections of the respiratory tract. Two years before admission he had been hospitalized suffering pneumonia. Serum protein electrophoresis revealed severe hypogammaglobulinemia(0.3 g/dl). Results of serum protein immunoelectrophoresiswere as follows: IgG 178 mg/dl (normal range, 6461,440 mg/dl), IgM 25 mg/dl (normal range, 55-200 mg/dl), and IgA 40 mg/dl (normal range, 65-350 mg/dl). Salivary IgA concentration was 21 mg/dl (normal range, 4.08 & 3.72 mg/dl). B and T lymphocyte rates in peripheral blood were normal, 7% and 71%, respectively. Antibody serum titres to rubella, cytomegalovirus, tetanus, and streptolysin 0 were absent or decreased. Lymphocyte reactions to phytohem-
From the Gastroenterology Service of the Departments of Medicine and Pathology. Hospital "12 de Octubre," Complutense University, Madrid, Spain. Address correspondence and reprint requests to Dr. G. Castellano Tortajada at Servicio de Gastroenterologia, Hospital Universitario "12 de Octubre", 28041 Madrid, Spain.
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J CIin Gastroenterol 1992;15(2):128-35.
Malignant Lymphoma of Jejunum with Common Variable Hypogammaglobulinemia and Diffuse Nodular Hyperplasia of the Small Intestine A Case Study and Literature Review G. Castellano, Ph.D., D. Moreno, Ph.D., 0. Galvao, M.D., c. Ballestin, M.D., F. Colina, Ph.D., M. Mollejo, M.D., J. D. Morillas, M.D., and J. A. Solis Herruzo, Ph.D.
We report a patient with common variable hypogammaglobulinemia and diffuse nodular lymphoid hyperplasia of the small intestine complicated by a jejunal malignant lymphoma. Immunopathological and histological studies showed a polymorphous centroblastic lymphoma with intracytoplasmaticIgM immunoglobulin and lambda light chains. Some mucosal nodules separate from the tumor mass showed atypical lymphoid cell populations similar to lymphoma cells, suggesting a transition between hyperplastic nodules and lymphoma nodules. Four similar cases, and six other patients with malignant lymphoma of the small intestine, associated with diffuse nodular lymphoid hyperplasia, but without immunodeficiency, have already been described. All these cases suggest that nodular lymphoid hyperplasia increases the risk of small intestine lymphoma. Key Words: Common variable hypogammaglobulinemia-Nodular lymphoid hyperplasia-Primary gut lymphomas.
The etiology of the small intestine primary lymphoma remains unknown and probably depends on several causes. An increased incidence of small intestine lymphoma has been found in celiac sprue (l),dermatitis herpetiformis (2), alpha-heavy chain disease (3), regional enteritis (4),and immune system dysfunction of different etiologies (congenital, autoimmune, postransplantation, and AIDS) (5-7). However, most small intestine lymphomas are not associated with any,of these problems (8)A high frequency of malignant epithelial and lymphoid tumors has been noted in common variable hypogammaglobulinemia (CVH) (9-1 1). Nevertheless, to the best of our knowledge, only four small intestinal lymphomas have been described (12-15), all associated with diffuse nodular lymphoid hyperplasia (DNLH). W e report here a new case of small intestine lymphoma in CVH with DNLH.
CASE REPORT A 39-year-old man had frequent bouts of diarrhea and recurrent infections of the respiratory tract. Two years before admission he had been hospitalized suffering pneumonia. Serum protein electrophoresis revealed severe hypogammaglobulinemia(0.3 g/dl). Results of serum protein immunoelectrophoresiswere as follows: IgG 178 mg/dl (normal range, 6461,440 mg/dl), IgM 25 mg/dl (normal range, 55-200 mg/dl), and IgA 40 mg/dl (normal range, 65-350 mg/dl). Salivary IgA concentration was 21 mg/dl (normal range, 4.08 & 3.72 mg/dl). B and T lymphocyte rates in peripheral blood were normal, 7% and 71%, respectively. Antibody serum titres to rubella, cytomegalovirus, tetanus, and streptolysin 0 were absent or decreased. Lymphocyte reactions to phytohem-
From the Gastroenterology Service of the Departments of Medicine and Pathology. Hospital "12 de Octubre," Complutense University, Madrid, Spain. Address correspondence and reprint requests to Dr. G. Castellano Tortajada at Servicio de Gastroenterologia, Hospital Universitario "12 de Octubre", 28041 Madrid, Spain.
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