Marshall-Smith syndrome: two case reports and a review of pulmonary manifestations.

Two patients with the Marshall-Smith syndrome are described. Both had significant and fatal respiratory distress attributable to this condition. Congenital, functional, and acquired abnormalities of the respiratory tract are described in nine of the 11 case reports in the literature and are characteristic of this syndrome as well as a primary cause of failure to thrive and death in these patients. Unusual immunologic findings in one of our two patients are the first to be reported in the Marshall-Smith syndrome. Quantitation of immune function in other patients with this condition will be helpful in determining the significance of these results. It is hoped that the etiology of the syndrome will be discovered as more cases are recognized and reported by pediatricians caring for infants with failure to thrive, advanced bone age, and chronic respiratory symptomatology.

Individual normal laboratory values. Preliminary observations.

A brief and preliminary outline is given describing a consecutive and continuing study of laboratory blood values of only 12 of 48 "normal healthy" subjects with only a few values given. The major emphasis is that of obtaining blood from each subject over a 12-week period to be repeated annually in order to determine individual values and in obtaining a chemical identification of each subject with the anticipation that more information will be available concerning the meaning and limiting parameters of "normal" biologic values. Such a study is made available through the application of modern advances in automation and the wide use of computers. It seems likely that some disorders can be discovered before clinically apparent, with the hope that consequent preventive measures and therapy may be more effective. The few values presented represent only a small part of those yet to be obtained. Much more work is needed in this study of normal values whose parameters must be further defined.

Use of immunologic markers in obscure hematologic disease.

Tumor cells from two patients with very different clinical diseases were evaluated using immunologic techniques. The first patient had a persistent T-cell lymphocytosis and other symptoms suggestive of T-cell malignancy. She also had an adnexal mass. Following surgical excision, the mass was identified as a benign teratoma that contained thymic tissue. The T-cell lymphocytosis subsided following removal of the mass. The second patient had leukemia, the cells of which were morphologically identified as myelomonocytic. Surface marker studies, however, showed the presence of monoclonal surface immunoglobulins. Enzymatic digestion of the cells showed that the antibodies were cytophilic and not produced by the tumor population. These studies emphasize the value of immunologic techniques in characterizing tumor cells.

Peripheral T-cell lymphoma. A clinicopathologic study of a morphologically diverse entity.

We analyzed the clinicopathologic features of 13 patients with immunologically confirmed peripheral T-cell lymphoma. The lymphomas were classified into poorly differentiated lymphocytic, mixed cell, and large cell types. Marked morphologic heterogeneity was noted within the mixed cell and large cell categories, and the various subtypes are described. Twelve of the 13 patients received multiagent chemotherapy. Only three of the nine patients with poorly differentiated or mixed cell lymphomas achieved a complete remission, and the median survival for this group was 11 months. In contrast, all three of the treated patients with large cell lymphomas achieved a complete remission, two of whom are alive without disease (14 and 29 months, respectively). Classification of peripheral T-cell lymphomas into lymphocytic, mixed cell, and large cell types, as well as further subclassification within the heterogeneous groups, is suggested so that pathologic features of prognostic significance can be identified.