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(Communicated by Saj iro MAKINO, M. J. A., May 12, 1982) It has been known that Klinefelter's syndrome is associated occasionally with myeloprolif erative disorders, particularly in a majority of cases with different forms of leukemia. The literature refers to only one case of polycythemia vera in association with this syndrome (Geraedts et al., 1980) . In this paper are reported the chromosomes of a male patient who had symptoms and signs of Klinef elter's syndrome with diabetes mellitus and developed later polycythemia vera.Case report. The patient was born in 1913 between a 48-yearold father and a 29-year-old mother. He married at the age of 26 years, but has no children. He is the third of five siblings, and all the siblings have children except the youngest brother who died in the World War II. There is no consanguinity in his family. Diabetes mellitus was diagnosed at the age of 50 years. At the age of 62 years, conj unctival congestion and erythema of the hands were noted. His red blood cell count was found to be markedly elevated at the age of 65 years. Then he was referred to our clinic due to polycythemia and diabetes mellitus.On physical examinations the patient was 171 cm tall, weighed 65 kg, and had an arm span of 175.5 cm (Fig. 1). His skin was deep red in color on the cheeks, lips, ears, tip of the nose and distal portions of the extremities. Bulbar and palpebral conjunctivae were markedly congested. He had bilateral gynecomastia and cubitus valgus, and a simian crease on the right hand. The axillary and pubic hair was scanty, and the testes were small measuring 2.5 by 1.5 cm on both sides. Hepatosplenomegaly was noted.Hematological examinations revealed RBC 938 x 104/cmm, Hb 24.0 g/dl, Ht 79.2% and WBC 12,400/cmm with 78% neutrophils and 8 % eosinophils. Platelet count was 7.2 x 104/cmm, while it was 45.3 x 104/cmm after a series of phlebotomy totaling 740 ml. Erythrocyte sedimentation rate was 0 mm in one hour. Blood chemistry

The literature indicates that the total loss of the short arm of the X chromosome causes full Turner's signs, such as short stature, gonadal dysgenesis, and many other stigmata characteristic of this syndrome."'2' However, the correlation between partial short arm deletions of the X chromosome and characteristic clinical signs has not yet been fully defined. The present paper concerns a 27-year-old female patient having an Xp-in region p11 (46,X,del(X)(gter-*p11:)) and Turner's signs. A brief review of clinical features in relation to chromosomal loss in the 46,XXp-cases is presented in reference to the relevant literature. Case report. The patient, C. I., was the 2nd of 2 siblings, and was born in 1951 after an uncomplicated pregnancy. The birth weight was 3.2 kg. The patient's mother and father were 30 and 35 years of age, respectively, at the birth of this patient. There is no consanguinity in them. The father is short, 153 cm in height, but is otherwise normal. The mother is 156 cm tall and the brother 174 cm tall. They are all in good health.

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