Myelodysplastic syndromes according to FAB and WHO classification. Single center experience.

The results of clinical and laboratory observations of 119 MDS patients divided acc. to FAB, and - after excluding RAEB-t and CMML groups -- of 95 patients divided accordingly to WHO classification are presented. The diagnosis of MDS was based on medical interview, physical examination, blood biochemistry, peripheral blood (PB) and bone marrow (BM) cytomorphology and cytochemistry, trephine biopsy and cytogenetic examination. All hematologic examinations were done according to routine methods. Cytogenetic analyses were carried out on BM cells from 24-48 h cultures in standard conditions. At least 15-20 GTG-banded metaphases were analyzed in every patient. The survival time (ST) of patients differed significantly between the FAB or WHO groups, with p=0.0004 for FAB and p=0.02 for WHO. The progression to AML was more common in less favorable groups, with p=0.0001 for FAB and p=0.00016 for WHO. The distribution of IPSS prognostic index among the groups showed statistically significant difference (p=0.0004 for FAB, and p=0.0001 for WHO), whereas the distribution of karyotypic abnormalities did not. However, in univariate analysis statistically significant influence on ST showed, beside the both classification systems: cytogenetics, the presence of blasts in PB, age and IPSS index. In multivariate analysis the sole independent prognostic factors were: PB blasts and cytogenetics. The authors conclude that the WHO classification offers a good prognostic tool for MDS patients. However, the karyotype and the presence of blasts in PB should always be taken into account.

Cladribine with prednisone versus chlorambucil with prednisone as first-line therapy in chronic lymphocytic leukemia: report of a prospective, randomized, multicenter trial.

The efficacy and toxicity of cladribine (2-CdA) + prednisone (P) versus chlorambucil (Chl) + P were compared in previously untreated patients with progressive or symptomatic chronic lymphocytic leukemia (CLL) in a randomized, multicenter prospective trial. Eligible patients were assigned to either 2-CdA 0.12 mg/kg per day in 2-hour infusions and P 30 mg/m(2) per day for 5 consecutive days or Chl 12 mg/m(2) per day and P 30 mg/m(2) per day for 7 consecutive days. Three courses were administered at 28-day intervals or longer if myelosuppression developed. The therapy was finished if complete response (CR) was achieved. Of 229 available patients 126 received 2-CdA+P and 103 received Chl+P as a first-line treatment. CR and overall response rates were significantly higher in the patients treated with 2-CdA+P (47% and 87%, respectively) than in the patients treated with Chl+P (12% and 57%, respectively) (P = .001). Progression-free survival was significantly longer in the 2-CdA-treated group (P = .01), but event-free survival was not statistically different. Thirteen percent of patients were refractory to 2-CdA+P and 43% to Chl+P (P = .001). Drug-induced neutropenia was more frequently observed during 2-CdA+P (23%) than Chl+P therapy (11%) (P = .02), but thrombocytopenia occurred with similar frequency in both groups (36% and 27%, respectively). Infections were seen more frequently in the 2-CdA+P-treated group (56%) than in the Chl+P-treated group (40%; P = .02). Death rates have so far been similar in patients treated with 2-CdA (20%) and with Chl (17%). The probability of overall survival calculated from Kaplan-Meier curves at 24 months was also similar for both groups (78% and 82%, respectively). (Blood. 2000;96:2723-2729)

Cladribine with or without prednisone in the treatment of previously treated and untreated B-cell chronic lymphocytic leukaemia - updated results of the multicentre study of 378 patients.

Between January 1992 and January 1999, we treated 378 B-chronic lymphocytic leukaemia (CLL) patients with cladribine (2-CdA), and 255 of the patients were also treated with prednisone. A total of 194 patients were previously untreated, and 184 had relapsed or refractory disease after previous other therapy. Complete response (CR) was obtained in 111 (29.4%) and partial response (PR) in 138 (36.5%) patients, giving an overall response (OR) rate of 65.9%. CR and OR were achieved more frequently in patients in whom 2-CdA was a first-line treatment (45.4% and 82.5% respectively) than in the pretreated group (12.5% and 48.4% respectively) (P < 0.0001). The median duration of OR for previously untreated patients was 14.7 months and for pretreated patients 13.5 months (P = 0.09). The median survival evaluated from the beginning of 2-CdA treatment was shorter in the pretreated group (16.3 months) than in the untreated group (19.4 months) (P < 0.0001). A total of 117 (63.9%) patients died in the pretreated group and 63 (32.6%) in the untreated group. In pretreated patients, 2-CdA + prednisone (P) and 2-CdA alone resulted in similar OR (51.0% and 45.0% respectively; P = 0.4). In contrast, in untreated patients, 2-CdA + P produced a higher OR (85.4%) than 2-CdA alone (72.1%) (P = 0.04). Infections and fever of unknown origin, observed in 91 (49.4%) pretreated and 74 (38.1%) untreated patients (P = 0.03), were the most frequent toxic effects. Our results indicate that 2-CdA is an effective, relatively well-tolerated drug, especially in previously untreated CLL.

2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia and hairy cell leukemia variant: 7-year experience in Poland.

Between January 1991 and December 1997, 103 patients, 97 with typical hairy cell leukemia (HCL) and 6 with HCL-variant (HCL-V) were treated with 2-chlorodeoxyadenosine (2-CdA) given as 2-h infusion for 5 consecutive d at a daily dose 0.12 mg/kg. To our knowledge this is the largest cohort of HCL patients treated with this type of regimen. Median follow-up amounted to 36 months. Fifty-six of 97 patients with typical HCL were newly diagnosed and 41 were relapsed after previous treatment. Splenectomy as a first-line therapy was performed in 23 patients and 18 remaining patients received prednisone, chlorambucil or interferon-alpha (IFN-alpha) alone or in combinations. Seventy-five (77.3%) patients entered CR and 18 (18.6%) achieved PR, giving an overall response rate of 95.9%. The mean time of first CR duration amounting to 32 months (range 3-72) did not correlate to the number of 2-CdA cycles. 2-CdA was equally effective in treatment of newly diagnosed patients and patients who relapsed after previous therapeutic procedures. Relapse of the disease occurred in 20 of 75 patients who achieved CR after 2-CdA and was usually manifested by very discrete changes in peripheral blood counts (neutropenia and/or relative lymphocytosis). The mean progression-free survival (PFS) time in this group was 37.4 (range 10-66) months. Ten of 20 relapsed patients were retreated with 2-CdA given an identical course to the first one. Seven patients entered second CR lasting 19+ (range 8-47) months and 3 experienced PR. This confirms the previous observations that 2-CdA gives no resistance to leukemic clone. Ten remaining patients have not required retreatment so far and remain in a good clinical and hematological state. The results of HCL-V treatment with 2-CdA were poor. Only 2 patients achieved PR and 4 patients did not respond to this drug. Seven patients (5 with typical HCL and 2 with HCL-V) died, 3 of causes unrelated to the disease. Second neoplasms were noted in 5 patients. 2-CdA-related side effects resulted mainly from myelosuppression and infectious complications. In conclusion we confirm the effectiveness of 2-CdA in inducing CR in patients with typical HCL, but this drug is unable to completely eradicate the leukemic clone which results in the relapse of the disease. The real incidence of the relapse rate may be underestimated unless bone marrow biopsy is performed. The results of our study indicate that a 2-h infusion of 2-CdA in HCL patients is at least as effective as a 24-h infusion but more convenient to the patients, and may be given on an outpatient basis.

[Evaluation of early treatment results in Hodgkin's disease during the cytostatic protocol containing methotrexate]. / Ocena wczesnych wyników leczenia choroby Hodgkina wedlug protokolu cytostatycznego zawierajacego metotreksat.

28 patients suffering from advanced Hodgkin's disease were treated according to protocol containing 7 cytostatic drugs including methotrexate and corticosteroids, but not cyclophosphamide, chlormethine or procarbazine. 22 patients i.e. 78.5% attained complete remission and 5 i.e. 17.8% a partial one. Drug tolerance was satisfactory: out of side-effects the most common was myelosuppression, especially granulocytopenia. Our early results were similar to those obtained with MOPP-like protocols, but early undesirable effects less often and of a smaller degree. Because the protocol has been aimed at avoiding late toxicity of cytostatics, its final evaluation will be possible in the future.

[Candidiasis of the bile ducts and liver in the course of acute leukemia]. / Kandydoza dróg zólciowych i watroby w przebiegu ostrej bialaczki.

The case of acute lymphoblastic leukemia complicated by candidiasis of liver and bile ducts is described. After treatment with amphotericin B, flucytosine and fluconazole jaundice and fever disappeared. Liver function improved and Candida count in bile decreased. The patient died of pulmonary infection six months later.

[Actinomycosis as a complication of blood diseases]. / Promienica jako powiklanie chorób krwi.

Two cases of actinomycosis in chronic myelogenous leukaemia (CML) and Hodgkin's disease (HD) are presented. The first patient with blastic crisis in CML had painful infiltration above and beneath the clavicular region, affecting the right humeral joint, what required differentiation from neoplastic infiltration. Specific infiltration, formation of fistuli and good response to antibiotic therapy suggested actinomycosis. Finally actinomycosis was diagnosed by microbiological and histopathological examination. The infection with actinomycosis in the other patient interfered with early diagnosis of coexistant Hodgkin's disease. The histopathologic examination of right cervical lymph nodes indicated the chronic inflammatory process. The subsequent histo pathologic examination of the axillary lymph node showed Hodgkin's disease, type LD. Culture from the exudating wound after lymph node excision was positive for actinomyces. Simultaneous treatment with cytostatics and antibiotics resulted in complete recovery of patient. In the above reported patients, actinomycosis complicated the course of CML and HD. The immune deficiency of the organism in both patients facilitated the development of the actinomycosis.

[Long-term (over 10-years) survival in Hodgkin's disease]. / Dlugotrwale (ponad 10-letnie) przezycie w chorobie Hodgkina.

61 patients with Hodgkin's disease, i.e., 40.9% of all HD pts hospitalized in the years 1970-1981 are more than 10 years survivors: 46 are alive and 15 died of underlying disease or its complications. The most important prognostic factor at diagnosis was clinical advancement and the form A or B of HD. Age and sex also influenced survival but to a lesser degree. Patients living in CCR were more likely to have MC histology than those with relapsing disease, who more often showed LD and LP type. Among 5 persons with second neoplasms four disclosed NS type of HD. No statistical differences in clinical prognostic factors were encountered between further alive and those who died after more than 10-yrs. Almost all patients were able to normally continue their familial and professional lives.

[Aspergillosis of the lymphatic nodes]. / Grzybica kropidlakowa (Aspergillosis) wezlów chlonnych.

The authors emphasize fungal lesions to the lymphatic nodes confirmed by the presence of Aspergillus flavus in blood and throat smear cultures (on Sabouraud's medium) and presence of A. flavus in cytological examination of biopsy from the lymphatic node, increased number of eosinophils in peripheral blood, and infiltration of eosinophils in bone marrow and lymphatic nodes. Aspergillosis coexisted with the infection with Candida albicans and S. aureus. The treatment of recurrent tonsillitis with antibiotics and also lowered granulocyte myeloperoxidase activity with increased production of O2 peroxide ion might predispose to such fungal infection.

[Neoplastic diseases of the hematopoietic system and ischemia of the lower limbs]. / Choroby nowotworowe ukladu krwiotwórczego a miazdzycowe niedokrwienie konczyn dolnych.

The possible interaction between hematopoietic neoplasms and ischemia of the lower limbs in patients with both pathologies was subjected to analysis. Anaemia, polycythemia, thrombocythemia, increased leucocytosis in the peripheral blood, and hyperuricemia exerted unfavourable effect on the blood flow through the arteries of the lower limbs. In some cases effective cytostatic treatment diminished the ischemia of the lower limbs. Interactions between various drugs used in the chronic treatment of both pathologies in the same patient was also examined.

[Disorders in the menstrual cycle and fertility of women with Hodgkin's disease]. / Zaburzenia miesiaczkowania i rozrodczosc kobiet chorych na chorobe hodgkina.

Sixty two women with Hodgkin's disease were questioned about disorders in their menstrual cycle and fertility. Menstrual cycle disorders were noted by 38 respondents, i.e. over 60%. Amenorrhoea persisted in 24 of these women. These disorders were significantly more frequent in patient over 30 year of age. There were 52 labours in the group of 38 women with menstrual cycle disorders prior to them, and 21 labours in a group of 24 women without any abnormalities. Hodgkin's disease was diagnosed in 6 patients during pregnancy: 2 of them were treated with irradiations on the upper part of the body while 4 remained untreated. After radiotherapy, there were 2 deliveries in women of the group with menstrual cycle disorders, and 9 deliveries in women without such disturbances. Answers to the supplied questionnaire did not provide any information on the congenital abnormalities in neonates.

[Attempt of conservative treatment efficiency evaluation of inhabitants of Wroclaw]. / Próba oceny efektywnosci leczenia zachowawczego mieszkanców Wroclawia.

2979 inhabitants of Wroclaw aged from 18-80 years, in this 1581 women and 1398 men, have been examined stomatologically. Clinical material has been collected and analysed, average values of D, M, F in women and men in 5 age groups have been calculated. Indicators D/DMF, M/DMF and M/DMF as well as percentage of people under examination with toothlessness and frequency they take advantage of stomatological care have been calculated. The results point out higher D/DMF medicinal demand in men than in women. Along with the age of persons under examination percentage of extracted teeth in the DMF, M/DMF values increases and the proportion of effective healing to the demand F/DMF decreases. Over 80% of persons under examination take occasionally advantage of stomatological care without pain symptoms.

[Relationship between caries resistance in permanent teeth of the Wroclaw school children and the length of time of drinking-water fluoridation]. / Relation entre la résistance des enfants des écoles de Wroclaw à l'éqard de le carie des dents permanentes.

The age at which carious permanent teeth are equally distributed between upper and lower jaw depends upon the caries resistance of the individual. This age was called the "characteristic age" of the population. It was found that water fluoridation in Wroclaw (1967) postponed this characteristic age by two years. In females, this age is earlier than in males. This method is useful in the appredication of the caries susceptibility of a population. The effect of water fluoridation upon this susceptibility was shown. Caries resistance was found to be higher in males.