Paul J. Kurtin

The (11;14)(q13;q32) translocation in multiple myeloma. A morphologic and immunohistochemical study.

We identified 24 cases of multiple myeloma with the t(11;14)(q13;q32). In 22 cases, the t(11;14)(q13;q32) was part of a complex karyotype, and in 2 cases it was an isolated abnormality. All patients had clinical and laboratory features consistent with multiple myeloma. The median degree of plasma cell involvement in the bone marrow was 60%, and in 10 cases, the plasma cells had a lymphoplasmacytoid appearance. Of the 24 cases, 21 had intermediate or high proliferative rates based on labeling index studies. Immunohistochemical studies performed on all bone marrow biopsy specimens showed strong cyclin D1 nuclear positivity in 19 cases. There also was strong cyclin D1 nuclear positivity found in 6 of 30 additional cases without the t(11;14)(q13;q32) demonstrated by routine cytogenetics. The t(11;14)(q13;q32) in multiple myeloma results in overexpression of the cyclin D1 protein, which can be demonstrated by immunohistochemical stain. The cyclin D1 stain results in the additional cases of multiple myeloma suggest that the t(11;14)(q13;q32) may be more common than previously thought and may be missed by routine cytogenetics, particularly if the proliferative rate is low.

Lymphomatoid papulosis: A clinical and histopathologic review of 53 cases with leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene rearrangement studies

BACKGROUND Lymphomatoid papulosis (LyP) is a recurrent hemorrhagic papular skin eruption with a clinically benign course and histopathologic features of lymphoma. OBJECTIVE AND METHODS To better characterize this disease, we studied 53 patients seen since 1965. RESULTS A lymphoproliferative malignancy developed within 2 to 36 years after onset of LyP in eight patients. Histologically, the dermis in LyP showed an infiltrate of large (type A) or small (type B) atypical lymphocytes. The large atypical cells (type A) stained with CD30 (Ber-H2). Seven of the patients in whom lymphoma developed had type A histologic features. DNA flow cytometry showed mainly a diploid pattern, except for two cases that showed aneuploidy. Five of 11 patients showed T-cell receptor (TCR) clonal gene rearrangements; lymphoma has not developed in these patients. One patient had a TCR rearrangement in a plaque of mycosis fungoides but not in the LyP lesion. CONCLUSION LyP is either a reactive skin condition or a localized lymphoid malignancy. Neither DNA flow cytometry nor TCR gene rearrangement can predict the 15% to 19% of patients in whom a lymphoma will develop. Continued observation of all patients is essential.

Amyloid-like Pulmonary Nodules, Including Localized Light-Chain Deposition Clinicopathologic Analysis of Three Cases

Amyloid-like pulmonary nodules have been described in patients with systemic light-chain deposition disease, but their significance in other clinical contexts is unknown. We examined biopsy specimens of amyloid-like pulmonary nodules from 3 women without systemic light-chain deposition disease. Patient 1 (aged 62 years) had multiple pulmonary nodules and underwent 2 separate lung biopsies, the first showing nodules composed of kappa light-chain deposits accompanied by low-grade lymphoplasmacytic lymphoma limited to the lung and the second, obtained after chemotherapy 9 months later, showing only residual nodules without persistent lymphoma. Patients 2 (aged 65 years) and 3 (aged 69 years) had asymptomatic solitary pulmonary nodules. In all cases, electron microscopic examination showed dense granular extracellular deposits without the fibrillary characteristics of amyloid. Amyloid-like nodules should be distinguished from nodular amyloidosis and, in some patients, might represent a localized form of light-chain deposition.

Lymphoma Involving the Esophagus

Fewer than 1% of all lymphomas involve the esophagus; however, lymphoma of the esophagus represents an important cause of dysphagia. This study reviewed all cases of biopsy-proven lymphoma involving the esophagus presenting at our institution between 1945 and 1992. Twenty-seven cases were identified. Three were primary esophageal lymphomas. Eleven percent of the cases represented Hodgkins disease. Eighty-nine percent were non-Hodgkins lymphoma. Eighty-nine percent of the patients experienced dysphagia. Eleven lymphomas (41%) were located at the gastroesophageal junction, while the other 17 were in the esophagus proper. Seven of these cases occurred at relapse. Three had mediastinal adenopathy with secondary esophageal involvement Morbidity included tracheoesophageal fistula in 22%, and surgical repair was performed in half of these cases. Vocal cord paralysis occurred in 22%, with minimal sequelae. Esophageal stricture was present in 30%, usually necessitating dilation. The presentation, diagnosis, and management of this problem are multidisciplinary.

Malignant lymphoma and leukemia with prominent ulceration: Clinicopathologic correlation of 33 cases

BACKGROUND The clinical and pathologic findings in patients with malignant lymphomas who presented with prominent cutaneous ulcers are described. OBJECTIVE Our purpose was to determine the histologic findings, type, and prognosis of lymphomas with cutaneous ulcers. METHODS Thirty-three patients (20 with cutaneous T-cell lymphomas, 10 with other non-Hodgkins lymphomas, and 3 with leukemia) were retrospectively studied. RESULTS All patients had a poor prognosis; 23 died within 9 months after the onset of the ulcers. Patients with non-Hodgkins lymphoma had a worse prognosis, had a higher incidence of systemic involvement, and more often had ulcers as an initial manifestation of lymphoma than did the patients with cutaneous T-cell lymphoma. The histopathologic findings ranged from a nonspecific inflammatory infiltrate to ulcers with marked adjacent epidermal epidermotropism to ulcers with an angiocentric infiltrate. CONCLUSION A variety of lymphomas may cause cutaneous ulceration. Adequate sampling of these ulcers is necessary for diagnosis. The average life expectancy after ulcer formation is 21 months.