Anovulation presumably due to the gonadotrophin-resistant ovary syndrome.

The clinical syndrome of the resistant ovary is described in a 24-yr-old woman (XX genotype) with secondary amenorrhea and primary infertility. She presented an increased secretion of gonadotrophins with a decreased secretion of estrogens. In the ovarian tissue only primary ovarian follicles and a thickened tunica albuginea were found. The elevated serum gonadotrophins could be further increased by the administration of exogenous LHRH and incompletely suppressed by exogenous estrogens (50 micrograms ethynylestradiol daily). However, serum LH concentration started with a further rise (positive feedback?) during this estrogen administration. Results of treatment with Cyclocur and ethynylestradiol (100 micrograms daily) in higher dosages are presented. Following discontinuation of the estradiol therapy regular menses resumed, which became ovulatory. The patient became pregnant 23 months after stopping the estradiol therapy.

[Benign histiocytosis: Rosai-Dorfman disease]. / Benigne histiocytose: ziekte van Rosai-Dorfman.

BACKGROUND: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease (RDD)) is a rare entity with pathological accumulation of specific, activated macrophages, mainly in cervical lymph nodes. However, in half of patients its primary presentation is extranodal. Its aetiology is unclear; several clues point in an immunological direction. Common clinical presentation is massive cervical lymphadenopathy, but nearly half of all patients suffer from extranodal manifestation at very diverse localizations. Diagnosis is based on histopathological findings: emperipolesis and S-100 positive histiocytes. Taking into account its benign character, caution in implementing therapy is recommended. Therapeutic options are limited to specific situations only. CASE DESCRIPTIONS: Two patients with known RDD presented at our outpatient clinic. A 75-year-old woman with two tumours in her right breast and an 83-year-old man with constipation - most likely due to a kidney tumour. Microscopic findings finally confirmed extranodal manifestation of RDD. CONCLUSION: Extranodal manifestations of RDD present in various forms and on history taking, physical examination and radiological imaging may resemble malignancies.

Cisplatin-based chemotherapy in advanced adenoid cystic carcinoma of the head and neck.

Nineteen patients, nine men and 10 women, with advanced adenoid cystic carcinoma (ACC), were treated with cisplatin either alone or in combination with doxorubicin and bleomycin. Median age was 51 years (range: 32-73 years). Two groups of patients were distinguished: Group 1 (N = 10) received single-agent cisplatin (50-120 mg/m2 IV every 4 weeks) for locoregional recurrence (N = 4), pulmonary metastases (N = 5), or as neoadjuvant therapy (N = 1). Five patients failed previous chemotherapy. No objective responses were observed, five patients showed stabilization of their disease for a median duration of 20 months (range: 3-50 months). Group 2 (N = 9) received a combination of cisplatin (20 mg/m2 IV on days 1-5), doxorubicin (50 mg/m2 IV on day 1), and bleomycin (30 mg IV on days 1-5), every 3 weeks. A complete remission (CR) was seen in one patient, lasting for 2 years, a partial remission (PR) in two patients (duration: 6 months and 6 years) (33%), and a stable disease (SD) in five patients (median duration: 15 months; range 3-24 months). One patient showed progression from the start. The observed toxicity was acceptable: dose reduction was required in five patients for myelosuppression or impairment of renal function; vomiting grade III (WHO) was seen in 10 patients. The median progression-free survival was 36 months (range: 7-77 months). Median overall survival was 81 months (range: 14-216 months). The role of cisplatin in this disease remains questionable.

Alterations in size, shape and osmotic behaviour of red cells after splenectomy: a study of their age dependence.

In whole blood from splenectomized subjects (n = 20), red cells showed a significant increase of mean surface area (MSA), mean cell volume (MCV), MSA/MCV-ratio and osmotic resistance, with the mean cell haemoglobin concentration (MCHC) being decreased. Studies on red cell populations of different cell age revealed that the increase of MSA affects younger and older cells, whereas the increase of MCV can mainly be ascribed to young cells with low density. The increased osmotic resistance is mainly determined by older cells due to a more favourable MSA/MCV-ratio. Shortly after splenectomy (n = 5) the MSA of younger and older cells increased, whereas the increase of MCV affected only young cells with a lowered density; moreover, the MSA/MCV-ratio increased in older cells in particular, resulting in a relatively greater increase of osmotic resistance. An impaired maturation of the reticulocyte may underlie the initial increase of MSA and MCV of young cells, but the present results contradict the current view that delayed maturation explains the changes in morphology and osmotic resistance of asplenic red cells.

Reticulocyte crisis after splenectomy: evidence for delayed red cell maturation?

In whole blood from splenectomized subjects (n = 8) in a steady state an increased number of reticulocytes was observed (14.0 +/- 7.8% versus 3.6 +/- 2.4% controls: p less than 0.05). Cell fractionation on density showed that reticulocytes, as in normals, are more or less confined to the least dense fraction; cell fractionation on volume revealed that reticulocytes form a heterogeneous cell population with diverging volume, but sharing a low density. Immediately after splenectomy (n = 4), a reticulocytosis is observed, mainly manifest in the top fractions (262.0 +/- 49.0% versus 40.0 +/- 32.4% preoperatively; p less than 0.01). This reticulocytosis is not due to pre- or postoperative complications, but is associated with the splenectomy itself and probably is the consequence of a delayed, but ultimately occurring red cell maturation. The increase of reticulocytes is not accompanied by significant alterations in cell morphology, as far as cell volume is concerned.

Vacuoles in red cells from splenectomized subjects originate during cell life: association with glycosylated haemoglobin?

Whole blood from splenectomized subjects (n = 8) contained a substantial percentage of vacuolated ('pitted') red cells (34.7 +/- 8.3%), while blood from controls revealed none. The percentage of haemoglobin A1 (HbA1) had increased significantly compared with controls (p less than 0.01). Fractionation on cell density revealed that the number of 'pitted' cells and the HbA1 percentage were associated with increased cell density. Fractionation on cell volume showed that 'pitted' cells are equally distributed in fractions with varying mean cell volume (MCV) and that decreasing MCV was associated with a linear rise of HbA1. It appeared that, shortly after splenectomy (n = 4), 'pits' develop early in cell life and that also older cells, after previous splenic contact, are capable of pit formation. A positive correlation found between the number of 'pitted' cells and the percentage of HbA1 might point to an impaired release of HbA1, manifest in the presence of 'pits'. The increased percentage of HbA1 in whole blood from splenectomized subjects may thus be explained.