[76-year old patient with suspected but not confirmed "idiopathic myelofibrosis"]. / 76-jähriger Patient mit nicht bestätigtem Verdacht auf "idiopathische Myelofibrose"

A 76-year old patient was referred to our Oncology Unit due to hepatosplenomegaly, anemia, thrombocytopenia, eosinophilia, elevation of lactat dehydrogenase and leucocytosis with occurrence of myeloid and erythroid precursors in the peripheral blood. Histopathological examination of the bone marrow and molecular genetics showing a c-kit-D816V-mutation confirmed the diagnosis of systemic mastocytosis without evidence of idiopathic myelofibrosis. After starting of prednisone treatment anemia, thromocytopenia and the patient's performance status rapidly improved.

The clinical benefit of pegylated liposomal doxorubicin in patients with metastatic breast cancer previously treated with conventional anthracyclines: a multicentre phase II trial.

This study evaluates the clinical benefit of pegylated liposomal doxorubicin (PLD) in patients with metastatic breast cancer (MBC), previously treated with conventional anthracyclines. Seventy-nine women with MBC previously treated with anthracyclines received PLD 50 mg m(-2) every 4 weeks. All patients were previously treated with chemotherapy and 30% of patients had > or =3 prior chemotherapies for metastatic disease. Patients were considered anthracycline resistant when they had disease progression on anthracycline therapy for MBC or within 6 months of adjuvant therapy. The overall clinical benefit rate (objective response+stable disease > or =24 weeks) was 24% (16.1% in patients with documented anthracycline resistance vs 29% in patients classified as having non-anthracycline-resistant disease). There was no difference with respect to the clinical benefit between patients who received PLD >12 months and those who received PLD < or =12 months since last anthracycline treatment for metastatic disease (clinical benefit 25 vs 24.1%, respectively). Median time to progression and overall survival were 3.6 and 12.3 months, respectively. The median duration of response was 12 months, and the median time to progression in patients with stable disease (any) was 9.5 months. Fourteen patients (17.7%) had a prolonged clinical benefit lasting > or =12 months. In conclusion, PLD was associated with an evident clinical benefit in anthracycline-pretreated patients with MBC.

Acute anterior myocardial infarction as first manifestation of acute myeloid leukemia.

A 42-year-old man was admitted with heavy retrosternal pain lasting 30 min. Electrocardiography showed typical signs of acute anterior myocardial infarction. The patient reported only attacks of coughing for a couple of days, and no serious diseases. The physical examination was normal. Laboratory tests showed a white blood cell count of 45/nl, platelet count of 58/nl, and hemoglobin of 14.4 g/dl. Blood chemistry showed elevated lactic dehydrogenase (413 U/l) but no elevation in creatine phosphokinase or glutamic-oxaloacetic transaminase. Therefore no thrombolysis was administered, but coronary angiography was performed. This showed a long-distance, subtotal thrombotic occlusion of the left anterior descending artery. After percutaneous transluminal coronary angioplasty and implantation of serial stents a normal perfusion of the artery was observed. The patient's blood and bone marrow films revealed acute myeloid leukemia FAB M2. Various conditions can cause a myocardial infarction in leukemias. We discuss the clinical management and the possible reasons for a subtotal thrombotic occlusion of the coronary artery.

[Recurrent pneumothorax in interstitial lung disease, anogenital and abdominal wall exanthema and secondary amenorrhea in a 20-year-old patient. Langerhans-cell histiocytosis]. / Rezidivierende Pneumothoraces bei interstitieller Lungenerkrankung, anogenitales und Bauchdeckenexanthem sowie sekundäre Amenorrhö bei einer 20-jährigen Patientin.Langerhans-Zell Histiocytose.

CASE REPORT: A 20-year-old female patient was admitted to the hospital because of a pneumothorax. A secondary amenorrhea was coexisting. On the abdominal wall and in the anogenital area a maculopapular exanthema was visible. Chest X-ray and HR-CT showed an interstitial lung disease with high probability of histiocytosis X which was confirmed by biopsies of lung and skin tissue. The cause of amenorrhea was a histiocytic infiltration of the pituitary stalk. Conventional "consensus-treatment" for adults with vinblastine, prednisone and etoposide did not prevent relapsing bilateral pneumothoraces. Treatment was successful with the protocol as proposed by the international study for children. This treatment is characterized by higher dosage and combination with 6-mercaptopurine.

[Pharyngeal tuberculosis as a differential diagnosis to carcinoma]. / Pharynxtuberkulose als Differentialdiagnose zum Karzinom.

The differential diagnosis of pharyngeal tumors includes malignomas as well as chronic inflammatory processes. Squamous cell carcinoma is the most prevalent malignoma of the pharynx, representing about 90% of all malignomas of the head and neck. Malignant lymphomas, lymphoepithelial tumors (Schmincke's tumor) and anaplastic carcinomas are less prevalent. Amelanotic melanoma, rhabdomyosarcoma and extramedullary plasmocytoma are rare malignomas of the pharynx. Infectious diseases may also be a cause of pharyngeal tumors which have been reported to be associated with mycobacterial infections, syphilis, leproma, malleus and anthrax. Sarcoidosis and Wegener's granulomatosis are chronic inflammatory diseases of unknown etiology. We report a case of a 65-year-old female with an 11-year history of a slowly progressing tumor of the nasopharynx who had been admitted to hospital with suspicion of a malignoma.

Recombinant human interferon-alpha in the treatment of angioimmunoblastic lymphadenopathy: results in 12 patients.

Peripheral T-cell lymphomas of the angioimmunoblastic lymphadenopathy (AILD) type roughly correspond to lymphogranulomatosis X (LgX). They are currently treated with prednisone, either as a single treatment or with combination chemotherapy. However, both approaches are associated with high risks in this usually elderly patient population (median age 64 years). While looking for therapeutic alternatives to avoid these problems, the efficacy of low dose recombinant interferon-alpha 2a was examined. Patients received 3 x 10(6) IU daily as a subcutaneous (s.c.) injection. Those who achieved a complete remission continued with a maintenance treatment of 3 x 10(6) IU s.c. three times per week. A total of 14 patients received interferon. Twelve were evaluable for response. Six received interferon as primary and six as secondary treatment after the failure of previous treatment with prednisone or chemotherapy. Complete remissions were achieved in four, partial remissions in another four of 12 patients, whereas in the remaining four patients no change or progressive disease was observed. The median remission duration was 3.5 months; the longest durations of complete remissions were 6+ and 7 months. It is concluded that low dose interferon-alpha is an effective and well tolerated drug in T-cell lymphomas of the AILD (LgX) type. It is useful for salvage treatment in patients with contraindications or refractory to combination chemotherapy. It may be useful in adjuvant or maintenance treatment.

[Hypercalcemia in florid pulmonary and cervical lymph node tuberculosis]. / Hypercalcämie bei florider Lungen- und Halslymphknotentuberkulose.

A 57-year-old man with a history of tuberculosis (TB of the knee-joint 44 years ago and open pulmonary TB six months ago with poor patient drug compliance) had lost 10 kg in weight during the preceding six months. In addition, firm lymph-node swellings with redness of the overlying skin and a skin fistula developed on the right side of the neck. There was mild hypercalcaemia (albumin-corrected serum calcium concentration 2.59 mmol/l) and hyperphosphataemia (2.0 mmol/l) with low-normal serum concentrations of parathormone and of 25-hydroxy-vitamin D3 (14 ng/ml). Serum 1,25-dihydroxy-vitamin D3 concentration was reduced (16 ng/l). The activity of osseous isoenzyme of alkaline phosphatase was normal (19 U/l) and skeletal scintigraphy revealed no abnormality. The cervical lymph-nodes were excised (histology: active caseous lymph-node TB). Tuberculostatic treatment was begun with daily doses of 0.3 g isoniazid, 0.45 g rifampicin, 1.2 g ethambutol and 1.5 g pyrazinamide. Serum calcium concentration rose to 3.22 mmol/l (albumin-corrected) in the first two weeks, even though the patient was on a low-calcium diet. The various concentrations subsequently decreased, but remained slightly elevated throughout the period of observation (11 weeks). This is thus a case of TB-associated hypercalcaemia which was not caused by 1,25-dihydroxy-vitamin D3 or bone destruction.

[Pseudohyperkalemia in the serum in reactive thrombocytosis and thrombocythemia]. / Pseudohyperkaliämie im Serum bei reaktiven Thrombozytosen und Thrombozythämien.

In 80 patients with different platelet counts serum and plasma potassium concentration was measured, 30 minutes after blood sampling and three and six hours of storage. Serum and plasma concentrations differed, depending on the platelet count. In reactive thrombocytosis the mean difference was 0.7 mmol/l, independently of the time since sampling. In myeloproliferative diseases and with platelet counts of more than 1000 X 10(9)/l is was time-dependent. The mean difference was between 0.8 and 1.8 mmol/l, in individual cases as much as 2.6 mmol/l. Plasma potassium levels were independent of time and platelet count. The possibility of pseudohyperkalaemia in serum must be kept in mind whenever the platelet count is increased, regardless of its cause. In case of doubt potassium must be measured in platelet-free plasma.