Search

Thirty-six patients (pts.) with thrombotic thrombocytopenic purpura (TTP) were treated between May 1990 and May 2003. There were 31 women and 5 men; the average age was 37 years. Twenty-five cases were idiopathic and 11 secondary (3 infection - related, 5 occurred during pregnancy and 3 were drug - associated). The mean lag period between the first symptoms and the diagnosis was 8.5 days (in 14 pts. £ 5; in 22 > 5). On diagnosis neurological symptoms were present in 31, bleeding in 33, fever in 21 and renal impairment in 27 patients. The mean hemoglobin was 67.5 g/L, the mean platelet count was 10´109/L, and the mean reticulocytosis was 17%. The mean serum LDH was 1457 IU. Treatment included plasma exchange (PE) in 24 pts. and only plasma infusions in 12 pts. There were 24 complete responders (20 on PE) and 12 deaths (4 on PE); PE significantly improved survival (p5 days), the secondary form of TTP and the absence of coma at presentation. The use of PE significantly improved survival. TTP is a severe disorder requiring early recognition and diagnosis in general medical care facilities, which should lead to the timely treatment with PE.

Cytogenetic analysis has proven to be a mandatory part of the diagnosis of myelodysplastic syndromes (MDS) as well as a major indicator for predicting clinical course and outcome. Aside from the 5q-syndrome, no specific clinico-cytogenetic entity has been reported. To determine the incidence and clinical significance of acquired abnormalities of chromosome 17 in adult primary MDS, we reviewed the cytogenetic features of 271 patients detected at our institution during a 10-year period. Clonal cytogenetic abnormalities were identified in 109 cases. Among them, abnormalities of chromosome 17 were identified in 13 patients (11.9%). Five patients had „single" defects, while in eight patients abnormalities of chromosome 17 were associated with other chromosomal rearrangements („complex" defects). After chromosomes 5,7,8 and 1, abnormalities of chromosome 17 were the most frequent chromosomal rearrangements in our patients with MDS. Following „single" defects of chromosome 17 were identified: del(17)(pl2) in two cases, and i(17)(q10), del(17)(q21;q23) and del(17)(ql2;q22) in one case each. Two patients with del(17p), one with RAEB-t and the other one with CMML, had an aggressive course of the disease with accelerated leukemic transformation and short survival. Patient with i(17q) had RARS subtype and died soon after diagnosis, while other two cases with interstitial deletions of the long arm of chromosome 17 had RAEB subtype and stable, no progressive course of the disease. Among „complex" karyotypes with abnormalities of chromosome 17 we identified der(l 7) in four, monosomy 17 in two, and del(17p) and i(17q) in one case each. Most of these patients transformed to acute leukemia and had very short survival. The results of this study suggest that abnormalities of chromosome 17 are frequent finding in MDS. Loss of genetic material in 17p, both in „single" and „complex" defects, seems to be closely related to poor prognosis of MDS patients.

If the spleen is not fixed within the left subphrenic space, it gradually passes into the lower abdomen, where is much more exposed to trauma. Torsion of the splenic pedicle can also occur, causing the infarct necessitating an immediate surgery. Venous stasis causes splenomegaly and sometimes secondary hypersplenism. The authors present 16.5-year old girl with torsion of the splenic pedicle of floating spleen for 720°: in spite of that, the patient had neither splenic infarct nor splenic vein thrombosis, possibly due to thrombocytopenia, but she had splenomegaly and secondary hypersplenism with pancytopenia causing bleeding, sideropenic anemia and mild jaundice. After treatment with iron, the patient underwent splenectomy which resulted in almost immediate rise of the number of all blood cells, and even thrombocytosis. The authors suggest early surgical treatment of the floating spleen, preferably splenopexy, before development of severe complications when splenectomy had to be performed in the majority of patients. Accessory spleens, if present, should be saved.