Leukemia research
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To increase the knowledge of the final phase of acute myeloid leukaemia (AML) a retrospective review of the medical and nursing records of 106 adult patients with AML who had died in 1995-1997 was made. A total of 27 patients were treated with curative intent at the time of death and 79 patients were prescribed palliative care. ⋯ In 54% of the morphine administration days, no information on the effect of given morphine treatment was registered. To give AML patients in the final phase, the best possible treatment, skills in palliative medicine and palliative care are important.
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A prognostic impact of WHO classification of myelodysplastic syndrome (MDS) was studied in a group of 103 primary MDS patients with refractory anemia (RA) according to French-American-British (FAB) classification. Median survival of 37 patients with RA according to WHO criteria of 85.2 months was significantly different from that in both 37 patients with refractory cytopenia with multilineage dysplasia (RCMD) (47.0 months, P=0.002) and 29 patients with 5q- abnormality diagnosed by routine chromosome banding (36.2 months, P=0.0002). A more detailed karyotype analysis with fluorescent in situ hybridization (FISH) techniques confirmed 5q deletion as a sole cytogenetic abnormality in only 12 out of 29 patients, in 4 patients 5q- was associated with complex abnormalities involving 5q region, 13 patients had 5q deletion combined with further karyotype abberations outside 5q. ⋯ RCMD represents a poor prognostic subgroup of MDS clearly distinct from pure RA mainly due to short survival connected with progressive bone marrow failure and increased risk of leukemic transformation. We also suggest to define 5q- syndrome as primary MDS of FAB type RA with 5q deletion as a sole cytogenetic abnormality confirmed by FISH analysis. This definition enabled us to discriminate 5q- patients with favorable prognosis similar as in RA from those with poor outcome associated with 5q- combined with complex abnormalities involving either 5q or regions outside 5q.
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Chronic lymphocytic leukemia (CLL) is recognized as a unique lymphoproliferative disorder of CD5(+) B cells. However, many published series of CLL included a subgroup of CD5(-) cases. CD5(-) B cell CLL is a topic of controversy and its nature and true incidence remain unclear. ⋯ Previous CD5(-) CLL may include false negatives due to heterogeneity of the intensity of CD5 expression, CD5(-) MLUS and variant CLL; the latter likely represents CLL in transformation. All the patients with MLUS were found to have a mild and non-progressing lymphocytosis with CD5(-) phenotype. These features may be used to differentiate them from CLL.
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Karyotyping is important for diagnosis and prognosis of myelodysplastic syndrome (MDS). Using fluorescence in situ hybridization (FISH) either mitotic or interphase cells can be analyzed and a higher number of cells can be screened. This study evaluated the effectiveness of FISH in detecting the most common chromosomal abnormalities [-5/del 5q/-7/+8/del 11q23 and -Y] in 40 patients with MDS. Karyotype detected abnormalities in 35.2% of the patients and FISH in 35%, while some abnormalities remained undetected by each approach but the association of both methods increased the detection rate up to 40%.
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Review Case Reports
Pulmonary alveolar proteinosis as a terminal complication in myelodysplastic syndromes: a report of four cases detected on autopsy.
Secondary pulmonary alveolar proteinosis (PAP) is one of the complications of hematologic malignancy and immunosuppressive diseases. We encountered four cases of myelodysplastic syndrome (MDS) associated with PAP detected on autopsy. They consisted of two refractory anemia (RA) and two patients with refractory anemia with excess blasts in transformation (RAEBt) at the time of MDS diagnosis, but all of them developed leukemic phase and were resistant to chemotherapy at the time of pulmonary episodes. ⋯ Previously, 69 patients with PAP associated with hematologic disorders have been reported, but there have been only seven cases with MDS, including our four patients. Of the 69 reported cases of PAP in hematologic malignancies, 24/63 (38%) informative patients with infection had fungal infections of the lung; 2/7 (29%) MDS cases had fungal infection. We should, therefore, pay careful attention to this possibility in cases of MDS with lung complications, including PAP, especially in patients in the leukemic phase of MDS.