[Quantification of iron level in the liver by MR imaging in patients treated for transfusion siderosis]. / Kvantitering af jernindholdet i levern ved MR-billeddannelse hos patienter i behandling for transfusionssiderose.

Patients with chronic blood transfusion requirements develop progressive iron overload, which is followed by organ damage in severe cases. Chemical determination of the liver iron concentration in liver biopsies is still regarded as the gold-standard for a precise determination of the degree of iron overload, but cannot be performed just for determination of the liver iron concentration alone due to the possible harmful side effects due to percutaneous liver biopsies. We have therefore validated a non-invasive MRI-technique based on the calculation of the ratio between the signal intensity (SIR) of the liver and skeletal muscle. We found a good correlation between the chemically determined liver iron concentration and the corresponding SIR-values (r2 = 0.98, p < 0.0001) low inter-day variation (2.9 +/- 2.7 mumol Fe/g) indicating that our non-invasive method is applicable for the determination of the liver iron concentration and may also be used for monitoring the efficacy of iron chelation by repeated measurements.

Clinical experience with recombinant factor VIIa in patients with thrombocytopenia.

Platelets play a central role in primary hemostasis. The role of the coagulation mechanism during early stages of hemostasis is less clear, although increasing evidence is emerging indicating the ultimate importance of the factor VII (FVII)-tissue factor-dependent coagulation system in providing the first thrombin molecules necessary for the platelet activation to occur. Supporting this, early fibrin formation has been reported to occur within the bleeding time wound and infusion of recombinant FVIIa (rFIIa) has been shown to shorten the bleeding time in rabbits. We have investigated whether infusion of rFVIIa would enhance fibrin formation in bleeding time wounds in patients with thrombocytopenia as reflected by a shortening of the bleeding time. A reduction of the bleeding time was found in 55/105 cases (52%). The decrease was significantly more pronounced when the platelet count exceeded 20 x 10(9)/l. With the exception of an anaphylactoid reaction in 1 patient, no major adverse reactions related to the study drug were observed. Nine infusions of rFVIIa were given to 8 thrombocytopenic patients with overt bleeding. One patient received two infusions. Bleeding decreased in all patients and stopped in 6 patients.

[Extramedullary manifestations among adult patients with acute lymphoblastic leukemia (ALL)]. / Ekstramedullaere manifestationer hos voksne patienter med akut lymfoblastleukaemi (ALL).

In 56 adult acute lymphoblastic leukemia (ALL) patients, 71% presented with extramedullary leukemic infiltration in lymphoid tissues (EML- spleen, liver, lymphnodes or thymus). EML was seen most often in patients with T-ALL (0.05 greater than p greater than 0.02) and in patients with high white blood counts (0.1 greater than p greater than 0.05). Surprisingly, these more often achieved complete remissions (0.1 greater than p greater than 0.05), of which both the duration and disease-free survival were longer. Thus, EML at diagnosis seems to be a favourable prognostic factor. At diagnosis, 23% of the patients had extramedullary leukemic infiltration in non-lymphoid tissue (EMIL-CNS, testis, skin, pleural cavities or gingiva). While more of these patients were of T-cell origin (61%, p less than 0.01), they were less likely to achieve CR, both the duration of remissions (p less than 0.01) as well as the disease free survival were shorter. Not unexpectedly, during the course of disease, the incidence of relapse localised at EMIL (the majority presenting in "sanctuary sites") increased, while that in the bone marrow decreased. Interestingly, in patients in CR presenting with EMIL, the first sign of relapse was unilateral peripheral facial paralysis in 60%. Finally, it should be stressed that the course of disease in patients presenting with simultaneous EML- and EMIL involvement was like that seen for EMIL patients. We conclude that while involvement of leukemia in EMIL represents a bad prognostic sign, the affection of leukemia in EML does not seem to confer a poorer prognosis, for adult ALL patients.