[Glycosylated hemoglobin (HbA1c) and acute hemolytic anemia]. / Glykosyleret haemoglobin (HbA1c) og akut haemolytisk anaemi.

The concentrations of glycosylated haemoglobin (HbA1c) were determined in eight patients with acute haemolytic anaemia and were found to be significantly lower than in a control group of 16 patients. A case history is presented in which HbA1c was employed as longitudinal parameter of haemolysis. It is concluded that in haemolytic anaemia in non-diabetic patients, HbA1c is a specific parameter of haemolysis which may be employed as a supplement to the traditional parameters of haemolysis.

[The chronic Philadelphia chromosome-negative myeloproliferative syndrome I. Pathogenesis and pathophysiology]. / Det kroniske Philadelphia-kromosom-negative myeloproliferative syndrom I. Patogenese og patofysiologi.

In polycythaemia vera (PV) the erythroid progenitors proliferate autonomously independently of the circulating erythropoietin. The progenitors are hypersensitive to various growth factors, including insulin-like growth factor 1, which inhibits apoptosis in erythroid and myeloid progenitor cells. No change has been found in the erythropoietin (EPO) receptor. Thrombopoietin (Tpo) regulates the production of haematopoietic progenitor cells, particularly of platelets. By inhibiting apoptosis, this growth factor may be responsible for the autonomous proliferation of the megakaryocyte cell lineage in PV and idiopathic myelofibrosis (IMF), which are featured by highly elevated circulating Tpo levels. Thrombopoietin may also be involved in the pathogenesis of myelofibrosis and development of extramedullary haematopoiesis. Both fibrogenesis and angiogenesis in the bone marrow, spleen, and liver develop secondary to the release of various growth-promoting factors from the megakaryocyte cell lineage. The lesion of the pluripotent stem cell in PV and IMF seems to imply several defects, including lack of or decreased expression of the Tpo receptor, alterations in the sensitivity of progenitor cells to various growth factors, and alterations in important gene systems (Bcl-2), which govern cell survival. Essential thrombocytosis seems to be a heterogeneous disease entity, as about 50% of the patients have polyclonal haematopoiesis.

[The chronic Philadelphia chromosome-negative myeloproliferative syndrome II. Clinical findings, diagnostics and treatment]. / Det kroniske Philadelphia-kromosom-negative myeloproliferative syndrom II. Klinik, diagnostik og behandling.

Polycythaemia vera (PV) and essential thrombocytosis (ET) are clinically characterised by non-specific neurologic symptoms, peripheral circulatory disturbances (acrocyanosis, wounds, erythromelalgia) or abdominal symptoms. The treatment of PV includes phlebotomy, antiaggregation and cytoreduction. In ET, the primary treatment is also low-dose aspirin except for patients presenting with a haemorrhagic diathesis. Hydroxyurea may be associated with an increased risk of acute leukaemia or myelodysplasia. Therefore alpha-interferon and anagrelide should be considered in younger patients. Early cytoreductive therapy is advocated in patients with idiopathic myelofibrosis (IMF) to inhibit further progression of bone marrow fibrosis and further expansion of myeloid metaplasia in the spleen and liver. Treatment with androgens (danazol) and glucocorticoids may improve severe anaemia and thrombocytopenia. In younger patients, allogeneic bone marrow transplantation should be considered.

[Acute thrombotic complication at the debute of the Philadelphia chromosome-negative myeloproliferative syndrome]. / Akut trombotisk komplikation ved debut af det Philadelphia-kromosom-negative myeloproliferative syndrom.

METHODS: We describe eight patients with a diagnosis of a chronic myeloproliferative disorder, characterised in most patients by severe thrombotic complications at the debut of the disease. RESULTS: The symptoms were life-threatening in seven patients: acute upper gastrointestinal haemorrhage from oesophageal varices in four, an acute abdominal catastrophy owing to mesenteric vein thrombosis with intestinal gangrene in two, and a large cerebral infarction, which was lethal, in one. The same patient also suffered a thrombosis of the axillary and subclavian veins. Neurological symptoms, with headache, visual disturbances, dizziness, and impaired memory, were initial cardinal symptoms. In two patients, explorative laparotomy was performed with intestinal resection owing to gangrene. One patient had a toe amputation. DISCUSSION: The above symptoms are explained by thrombosis in the microcirculation because of thrombocytosis and circulating platelet aggregates. In patients with polycythaemia vera, the elevated haematocrit contributes significantly to the impaired microcirculation. Early diagnosis and management of these disorders are of utmost importance to prevent the potentially life-threatening complications described above.

Serum concentration of cathodic trypsin-like immunoreactivity and pancreatic isoamylase in insulin-dependent diabetes mellitus.

The relationship between diabetes mellitus and the exocrine pancreatic function was evaluated in 29 patients with insulin-dependent diabetes mellitus by measuring cathodic trypsin-like immunoreactivity (TLI) and the enzymatic activity of pancreatic isoamylase in serum before and 90 min after breakfast and insulin. Thirty healthy subjects served as reference group. Median fasting serum concentrations in the diabetic subjects were significantly lower than in the reference subjects: for TLI 143 and 299 micrograms trypsin standard/l and for pancreatic isoamylase 43 and 101 U/l, respectively (p less than 0.001 for both). A positive correlation between TLI and pancreatic isoamylase was present in the diabetics (Sperman's rho = 0.56, p less than 0.01). The serum concentrations of TLI and pancreatic isoamylase were not related to the duration of diabetes, daily insulin dose or glycemic control measured by blood glucose and total glycosylated hemoglobins. Cathodic TLI and pancreatic isoamylase in serum were not influenced by food and insulin.