Hematopathological features of acute erythremia (morbus Di Guglielmo). A contribution to the classification and differential diagnosis of erythroid neoplasias.

Classification and differential diagnosis of erythroid neoplasias still are a matter of discussion. Eleven cases of primary acute erythremia were diagnosed between 1981 and 1984 at the Institute of Pathology, University of Kiel. Erythremia represented 0.5% of all hematological diagnoses and 1.0% of the myeloproliferative disorders. The male-to-female ratio was 1:1. Incidence peaked in the 7th decade. Evaluation of clinical data, of cytological and histological findings in blood and bone marrow, and of occasional immunophenotyping of blast cells (anti-glycophorin A+) revealed two variants of acute erythremia: a first, blastic one and a second, more differentiated form. Acute erythremia must be strictly distinguished from mixed erythroid/myeloid erythroleukemia and from secondary erythroid neoplasias, especially the erythremic 'blast crisis' of chronic myeloid leukemia or polycythemia vera rubra. Distinguishing the myelodysplastic variant of sideroblastic anemia from anerythremic acute erythremia can be extremely difficult. We discuss the differential diagnosis and classification of erythroid neoplasias based upon reproducible hematological criteria to facilitate the gathering and comparison of epidemiological and clinical data on these rare malignancies.

Lymphoepithelioid lymphoma (Lennert's lymphoma) is a monoclonal proliferation of helper/inducer T cells.

Lymphoepithelioid lymphoma (Lennert's lymphoma) was first described as a special variant of Hodgkin's disease. This lesion is characterized by a high percentage of epithelioid and T cells and rarely contains the classical Hodgkin's/Reed-Sternberg cells. Cytogenetic abnormalities indicate that Lennert's lymphoma is of T cell origin. In the present study, immunohistochemical investigation of four cases of Lennert's lymphoma revealed two major cell populations of T cells that predominantly express the helper-inducer phenotype and Ki-M6- and Ki-M8-positive macrophages and epithelioid cells. Double-staining experiments for the detection of cell surface antigens and the proliferation-associated antigen Ki67 showed that only the CD4-positive cells (helper-inducer T cells) were proliferating. Examination of the DNA of these Lennert's lymphoma samples also indicated that monoclonal rearrangement of the T cell receptor beta-chain genes has occurred, whereas the immunoglobulin heavy- and kappa-chain genes remained in germline configuration. Our results strongly suggest that Lennert's lymphoma is a CD4-positive T cell lymphoma.

[Papillary adenocarcinoma of the vocal cord--first case in the literature]. / Papilläres Adenokarzinom des Stimmbands--der erste Fall in der Literatur.

The first case of a papillary adenocarcinoma of the vocal cord is presented. The patient was a 63-year-old man: he was treated by a frontolateral hemilaryngectomy. The patient is alive and well with no evidence of disease 5 1/2 years later.

Germinal center derived malignant lymphoma in cystadenolymphoma.

Two cases of malignant non-Hodgkin's lymphoma arising in an Albrecht-Arzt-tumour are reported. In the first case a centroblastic-centrocytic lymphoma in a palatinal cystadenolymphoma of a 64-year-old female is described. In the other case a centroblastic lymphoma developed in an Albrecht-Arzt-tumor of the submandibular region in an 82-year-old man. The occurrence of a high-grade malignant lymphoma in cystadenolymphoma has not been reported in the literature so far.

Adenosquamous carcinoma of the ileum. Report of a case and review of the literature.

Adenosquamous carcinomas of the intestine are rare tumours, especially those occurring in the small bowel. We report the first case of a metastasizing adenosquamous carcinoma of the ileum in a 74 year old male. The tumour mainly consisted of squamous cells, many of which reacted with antikeratin antibody. A review of the pertinent literature is given.

Soft tissue sarcoma as second malignant lesion after therapy for Hodgkin's disease. Report of two cases and review of the literature.

Two cases of soft tissue sarcoma following treatment of nodular sclerosing Hodgkin's disease are described. One patient developed a malignant schwannoma after radiotherapy, the other was diagnosed as having a malignant fibrous histiocytoma after treatment with radiation and chemotherapy. Both secondary malignancies arose within the irradiated field after a latent period ranging from 9.5 years in the first to 21 years in the second case. A review of the pertinent literature is given and previous reports of malignant tumors and leukemias following therapy for Hodgkin's disease are summarized.

Different myosins in myoid and entodermal reticular epithelial cells of the thymus. An immunocytochemical study using specific antibodies against striated and smooth muscle myosin.

A sensitive immunofluorescence microscopical technique employing specific antibodies against highly purified actin and (smooth muscle type) myosin from chicken gizzard and myosin from human striated muscle was used to localize these contractile proteins in the thymus of rat, guinea-pig, cat and chicken. Myoid cells were seen to react with antibodies to striated, but not to smooth muscle type myosin, whereas reticular epithelial cells contained smooth, but not striated type myosin. Actin immunoreactivity was found in both myoid and reticular epithelial cells.