Persistent pigmented purpuric eruption associated with mycosis fungoides: a case report and review of the literature.

A purpuric eruption may be an unusual early manifestation of mycosis fungoides (MF). On the other hand, persistent pigmented purpuric dermatoses (PPPD) may, occasionally, evolve to cutaneous T-cell lymphoma. Coexistence of these two conditions has been reported, but it is extremely rare. We present the case of an elderly woman with a long-standing pruritic, pigmented purpuric eruption. On 1-year follow-up, histological features suggesting early MF were observed and molecular analysis of the rearrangement of T-cell receptor genes revealed clonality. Our patient may represent a case of PPPD evolving to MF, a case of MF clinically featuring PPPD, or an intermediate condition in a nosological continuity extending from PPPD to MF. A persistent pigmented purpuric eruption may rarely be a harbinger of cutaneous T-cell lymphoma. Therefore, vigilant long-term follow-up of PPPD is highly recommended.

A novel chromosomal abnormality involving chromosomes 2 and 18 in a patient with myelodysplastic syndrome.

Cytogenetic analysis of bone marrow cells from a patient with myelodysplastic syndrome associated with eosinophilia showed a complex translocation with a 46,XY,t(2;18;2)(p23;q11;q32) karyotype. The patient has refractory anemia (RA) according to the French-American-British Cooperative Group (FAB) classification, and after 90 months of follow-up he shows no evidence of leukemic transformation. This chromosomal abnormality has not been previously described in myelodysplastic syndromes and may be associated with good prognosis as the patient has been stable for a long time.

HLA antigens in Burger's disease.

The HLA A and B types of 20 Greek patients with thromboangiitis obliterans (TAO) were studied and compared with a panel of 400 controls. A nonstatistically significant increase in the frequencies of HLA B5, B7 and A9 antigens was found. These antigens were found to be associated with a relative risk of 3.47, 3.24 and 2.07 respectively. These findings are partially in agreement with those of a joint English-Swiss study which also found a negative association with B12 antigens; in the Greek population, also of European origin, a negative association was observed with the B8 antigen.

The effect of recombinant alpha-interferon on natural killer cell activity and clinical course in patients with myelodysplastic syndrome.

Thirteen patients with myelodysplastic syndrome (MDS) were included in this study and consented to treatment with recombinant alpha-interferon (a-IFN). These patients were subclassified: six as RAEB, one as RAEB-T and six as CMML. T-cell subsets and natural killer cells were identified in the peripheral blood with the use of monoclonal antibodies and natural killer cell activity (NKa) was assayed before, during and after a-INF treatment. The treatment schedule consisted of 2.0 MU/m2 sc t.i.w. continuously for the three months. Prior to treatment, NKa was found decreased in 11 of 13 patients as compared to that of normal individuals. Following a-IFN administration, a rise of NKa was observed in eight of the eleven patients. In those who responded, a-IFN was continued for 1 to 21 months. Alpha-IFN treatment was myelosuppressive for most of the patients, but transient increase of the number of neutrophils and platelets was observed in 3 and of the reticulocytes in one patient. Disease progression was recorded in 9/13 patients (69%) at a median time of 17.3 months. The median overall survival was 30.5 months (range 7.5 to 65+ months). No evidence of a relationship was found between the rise in Nka and the limited clinical improvement observed. Two NKa responders under continuous a-IFN treatment are in stable clinical condition for 36+ and 65+ months. The study provides only limited evidence that a-IFN may improve the clinical course of patients with MDS.

Chronic lupus peritonitis with ascites.

A 28 year old woman with systemic lupus erythematosus who developed chronic lupus peritonitis and ascites is described. Lupus peritonitis appeared with abdominal fullness, postprandial abdominal discomfort, and painless ascites. Four months later the patient developed vertigo, headaches, visual disturbances, serositis, and glomerulonephritis. Lupus peritonitis and the other disease manifestations responded to treatment with intravenous pulse methylprednisolone (four 1 g/m2 injections at one week intervals), oral azathioprine (200 mg daily), and diuretics.

alpha Interferon treatment of essential thrombocythaemia and other myeloproliferative disorders with excessive thrombocytosis.

The effect of recombinant interferon alfa-2b on platelet count, thrombocytosis-associated symptoms and marrow fibrosis was studied in 18 patients with myeloproliferative diseases and associated thrombocytosis (nine with essential thrombocythaemia, three with polycythaemia vera, three with myelofibrosis and three with chronic myelogenous leukaemia). A reduction of the platelet count below 600 x 10(9)/L was achieved in 94%, and below 400 x 10(9)/L in 77% of the patients within 8 to 330 days of treatment. The selective thrombocytosis-reducing effect of alpha interferon was maintained for long periods of time in most patients without serious side effects. Thrombocytosis-associated symptoms were relieved once the number of platelets was reduced to near normal levels. Marrow reticulin content was found to be reduced after treatment in two of the seven patients studied. Side effects of alpha interferon were flu-like symptoms, which usually subsided within 7 days of treatment.

Randomized clinical trial comparing ceftriaxone/amikacin versus ceftazidime/amikacin as initial therapy of febrile episodes in neutropenic patients.

Neutropenic patients with underlying hematologic (usually malignant) diseases were randomized to receive either 2 g ceftriaxone once daily +0.5 g amikacin or 2 g ceftazidime twice daily +0.5 g amikacin b.i.d. when fever was higher than 38 degrees C and granulocyte counts less than 0.5 x 10(9)/l. 25 patients were included in each treatment group. Successful outcome of treatment was observed in 28 (13/15) and in an additional 5 (2/3) patients after modification of the therapy. Tolerability was excellent in both groups.

Rheumatoid arthritis with periarticular calcifications.

A rare case of rheumatoid arthritis with periarticular calcifications, lung involvement, pericarditis, nodules, Sjögren's syndrome and various drug side-effects is described. Over a period of more than 10 years of observation, the patient's arthritis has been characterized by remissions and exacerbations.

Late-onset systemic lupus erythematosus presenting as polymyalgia rheumatica.

Two patients (male, 60 and 66 years of age) who developed systemic lupus erythematosus (SLE) in the 6th decade are described. Both patients presented with a polymyalgia rheumatica (PMR) syndrome. In both cases there was an underlying muscle involvement (nonspecific in the first case and true myositis in the second case) as well as findings compatible with nonclassic type of temporal arteritis.

Tropical pyomyositis in a Greek adult.

A case of tropical pyomyositis in a 24-year-old Greek is reported. The patient presented with high fever and swelling of the left thigh, generalized lymphadenopathy and multiple infiltrations in both lung fields on X-ray of the chest. Multifocal muscle abscesses were detected by CT scan of the left thigh and gluteal area. Staphylococcus aureus was identified in cultures of the purulent material which was surgically drained. The patient was subsequently treated with appropriate antibiotics. Lack of familiarity with this disease caused diagnostic confusion and delayed the initiation of treatment.

HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks.

The distribution frequency of HLA-A and B antigens was determined in 24 Greek individuals with Adamantiades-Behçet's disease (A-BD) as well as in 280 controls. The standard NIH technique was used for tissue typing. There was an increased incidence of HLA-B5 in the patients group (75% compared to controls 32%). The calculated P corrected value was Pc less than 0.02, the relative risk (RR) was 6.23 and the aetiological fraction (EF) 0.68. This study agrees with the reported association between A-BD and HLA-B5 in other populations from the Mediterranean basin, the Middle East and Far East.