Disseminated form of the Kaposi sarcoma in HIV-negative patient associated with Hodgkin's lymphoma.

We report a case of a 35-year-old, non-HIV-infected male diagnosed simultaneously with a disseminated form of Kaposi's sarcoma (KS; skin, stomach and colon are involved) and Hodgkin's lymphoma. There is no sign of changes in the immune status, but three herpes viruses were detected in the patient's blood (EBV, HHV6 and HHV8). He received ABVD chemotherapy and achieved complete metabolic remission for Hodgkin's lymphoma. Moreover, the signs of the disseminated KS were resolved. Our observations indicate that a combination of distinct types of viruses may play an important role in triggering the development of angio- and lymphoproliferative disorders in the same person. In addition, treatment with chemotherapy cycles, which included doxorubicin and vinblastine, led to the stable remission of both diseases.

[Incidence of hepatitis B virus infection in patients with blood disease].

AIM: To define incidence of HBV infection in patients with blood diseases caused by blood components transfusion; correlation between infection rate and blood disease nosological entity, intensity of hemoreplacement therapy, time of hepatitis B incubation period in patients with hematological malignancies after the diagnosis and initiation of polychemotherapy (PCT). MATERIAL AND METHODS: In 2000-2007 a prospective clinicoepidemiological trial was made to detect markers of HBV infection among 303 patients 15 to 76 years of age treated in the department of acute leukemia chemotherapy of N.N. Burdenko Military Hospital for acute lymphoid and myeloblastic leukemia, chronic myeloid leukemia in a blastic crisis, myelodysplastic syndrome in blast transformation, lymphoproliferative diseases with bone marrow affection. Statistic processing was performed with standard methods. RESULTS: HBV infection markers were detected in 30 (9.9%) of 303 examinees. Among the infected patients there were 16 (53.4%) patients with different variants of acute myeloblastic leukemia, 12 (40.0%) with different immunophenotypes of acute lymphoblastic leukemia, 1 (3.3%) patient with acute biphenotypical leukemia and 1 (3.3%) with lymphoma/leukemia. HBV infection was registered in patients 2 to 32 months after the beginning of the treatment. Most of the patients - 23 (74%) of 30 - were infected with HBV within the first year after hematological diagnosis and PCT induction course. HBV was diagnosed within treatment year two in 5 (16%) patients and within year three after PCT in 3 (10%). CONCLUSION: High incidence of HBV infection in patients with hematological malignancies points to a high epidemiological risk of hemoreplacement therapy, unsatisfactory quality of donor blood testing and necessity of updating methods of donor infection detection. To lower the risk of HBV infection in patients with hematological malignancies it is necessary to perform vaccine prophylaxis of hepatitis B before PCT.

[A case of primary amyloidosis].

Diagnostics of primary (AL) amyloidosis is difficult enough; treatment of this disease in not less difficult or more adequate. Because of similarity of the pathogenesis of AL-amyloidosis and that of multiple myeloma, similar therapeutic regimens, directed towards depression of plasma cell dyscrasia, are used in both cases: administration of melphalan in various doses together with prednisolone, administration of vincristine, adriablastine and dexamethasone, as well as high-dose chemotherapy with melphalan and autologic stem cell transplantation. This therapeutic approach makes it possible to reach clinico-laboratory remission and prolong the life of patients with AL-amyloidosis. The article contains a case description of a patient with AL-amyloidosis, who underwent a successful high-dose melphalan therapy with subsequent autologic stem cell transplantation.

[Acute lymphoblastic leukemias with aberrations of BCR-ABL genes].

AIM: To develop an original therapeutic strategy in Ph-positive acute lymphoblastic leukemia (ALL). MATERIAL AND METHODS: In November 2001 Hematological Research Center (HRC) initiated the study of chimeric BCR-ABL gene. During the first stage of the study (November 2001-July 2004), 18 primary ALL patients were recruited in HRC, from July 2004 to January 2005--16 patients in HRC, N.N. Burdenko Central Military Hospital, regional Samara hospital. The diagnosis of Ph-positive ALL was established in detection of translocation t(9;22) by standard cytogenetic test or fluorescent hibridization in situ with double signal (D-FISH), or by polymerase chain reaction with reverse transcription (RT-PCR). In detection of aberration of BCR-ABL gene the patients received stem hemopoietic cells, from June 2004 imatinib was added to chemotherapy in the period of induction and consolidation. RESULTS: Incidence rate of BCR-ABL-positive ALL by standard cytogenetic test and D-FISH makes up 20%, by RT-PCR--25%. Differences in chimeric transcripts detectability by different methods may be explained by different sensitivity of the methods. Complete hematological remissions were achieved in the majority of the patients (6 of 8) irrespective of imatinib administration. Achievement of molecular remission in BCR-ABL-positive ALL occurs also in standard chemotherapy but molecular remissions begin 2-4 months later than clinicohematological ones. CONCLUSION: In using imatinib combination with chemotherapy, molecular remission can be achieved simultaneously with hematological one. Long-term results will be analysed later.

[The condition of intestinal microflora in patients with hematologic tumors receiving polychemotherapy].

The subjects of the study (105 patients with blood system tumors), were divided into 4 groups. The patients of the first group received only cytostatic therapy. The subjects of the second, third and fourth groups were administered a combination of cytostatic and antibacterial agents. In addition to this, the patients of the third and fourth groups received prebiotic preparations as means of corrective therapy. The results obtained by measurement of short-chain fatty acids (SCFA) in feces by means of liquid-gas chromatography were used as criteria for evaluation of the therapy influence on intestinal microflora. The study shows that the cytostatic and combined treatment worsen the disbalance between aerobe and anaerobe populations, which manifests in SCFA spectrum alteration. Use of prebiotic preparations results in improvement of intestinal microflora condition or prevents the aggravation of its disturbance caused by the treatment of the fundamental illness, which becomes apparent in stabilization or normalization of SCFA profile in feces. Study of SCFA makes it possible to monitor microbiocenosis, adjust corrective therapy individually and evaluate its effectiveness.

[Choice of therapy and overall survival in patients with chronic myeloproliferative diseases]. / Vybor terapii i obshchaia vyzhivaemost' bol'nykh khronicheskimi mieloproliferativnymi zabolevaniiami.

A retrospective analysis of 106 case histories of primary chronic myeloproliferative diseases (CMPD) was undertaken: idiopathic myelofibrosis--71 (67%), polycythemia vera--29 (27.3%), and essential thrombocythemia--6 (5.7%), median age--65 years (26-84 yrs). Hydroxyurea and myelosan were mostly used as cytostatic drugs while erythrocyte mass transfusions and hemoexfusions (phlebotomy)--for life-support. Median overall survival in patients untreated with cytostatics was 95.2 years as compared with 156 months in recipients of such drugs. Survival rates in all CMPD patients with hypocellular bone marrow who had received cytostatics were lower than in those with normal or hypercellular marrow (p=0.005). Cytostatic therapy had either no impact on survival in patients with hypocellular bone marrow or was followed by decrease. Among CMPD patients who had received erythrocyte mass transfusions survival rates were significantly lower than in intact ones (p=0.0009). Median overall survival in patients receiving hemoexfusions was 193.6 months, as compared with 110.3 months in intact ones (p=0.008). Our data may be useful in selecting therapy for CMPD.

[Low-dose cytosine-arabinoside (Ara-C) therapy for chronic myeloid leukemia]. / Primenenie malykh doz tsitozin-arabinozida (Ara-C) dlia lecheniia bol'nykh khronicheskim mieloleikozom.

The effects of low doses of cytosine-arabinoside (Ara-C) were studied in 17 patients with chronic myeloid leukemia: chronic, resistant to IFN and hydroxyurea therapy (including 4 cases of advanced chronic disease)--7, and tumor progression--10. Hematologic effect was recorded in 7 chronic patients tolerant to hydroxyurea and alpha-interferon therapy. Among 10 cases of tumor progression, chronic stage II was observed in 3, stabilization (tumor progression short of blastic crisis)--5, and without effect--1. Low-dose Ara-C treatment was considered effective in 15 (88%) out of 17 patients.

[Low doses of alpha-interferon preparations in the treatment of patients with chronic myeloleukemia]. / Malye dozy preparatov al'fa-interferona v lechenii bol'nykh khronicheskim mieloleikozom.

AIM: To compare the effects of low-dose alpha-interferons with those of cytostatics (hydroxyurea or myelosan) on survival of patients and duration of chronic phase of chronic myeloid leukemia (CML). MATERIAL AND METHODS: 107 CML patients were divided into two groups. 28 patients (15 males and 13 females) aged 17-59 entered group treated with alpha-interferon drugs. 79 control patients (35 males and 44 females) aged 15-79 received standard chemotherapy (hydroxyurea or myelosan). RESULTS: 3-year survival in the study group and controls was 94 and 67.5%, respectively. 5-year survival--70.8 and 28.9%, respectively. The survival medians for the groups were 66 and 48 months, respectively. 36 months after CML diagnosis, chronic phase of the disease still continued in 90.2% of the study group patients and in 53.4% of patients on chemotherapy. 54 months after the diagnosis the chronic phase was registered in 56.5 and 24% of patients, respectively. The medians made up 54 and 39 months, respectively. CONCLUSION: Treatment of CML with low-dose alpha-interferons increases duration of the CML chronic phase and survival of CML patients.

[Alpha-interferons in the treatment of patients with chronic myeloid leukemia]. / Al'fa-interferony v lechenii bol'nykh khronicheskim mieloleikozom.

Chronic disease duration and survival have been investigated in three groups of patients suffering chronic myeloid leukemia (CML). The first group included 13 patients on alpha-interferons 6-9 mln MU/24 h (mean dose--48 mln MU/week). 31 patients received 2 mln MU/m2/24 h; mean weekly dose--15 x 10(6) MU. Standard chemotherapy was given to another 79 patients (group III). Actual survival and chronic disease duration were computed after Kaplan-Meyer: 4-year survival in group I--88%; group II--85.6% and group III--54%. Five-year survival in patients who had received standard or lower doses of alpha-interferon was 78.7%; chemotherapy alone--28.9%. Median survival in alpha-interferon-treated patients was 66 months; chemotherapy--48 months. After standard alpha-interferon, chronic disease three years after CML diagnosis was in 87% of those treated with standard alpha-interferon, 89% of those receiving lower doses of the drug and 53.4% of chemotherapy-treated patients. After 4 years, chronic disease was registered in 75.5% (alpha-interferon)--74.8% in group I and 72.9% in group II, and in 34.4% of patients treated with myelosan or hydroxyurea. Median chronic stage duration after interferon was 51 months and 39 months in group III, hence, both standard and lower doses of alpha-interferon prolong chronic disease and improve survival in CML patients.

[The efficacy of treatment with native lymphoblast alpha-interferon (Wellferon) in patients with chronic myeloleukemia]. / Effektivnost' lecheniia nativnym limfoblastoidnym alfa-interferonom (vellferonom) bol'nykh khronicheskim mieloleikozom.

Data are submitted on treatment with vellferon of patients with chronic myeloid leukemia (CML) in the chronic phase of the illness. The data obtained suggest that vellferon has a pronounced therapeutic effect in CML patients. The drug has a positive effect on both the clinical- and hematological status and cytogenetic changes in bone marrow cells. After 3-month treatment of patients with vellferon the sizes of the liver and spleen returned to normal as did the peripheral blood leucocyte and platelet counts. Two patients revealed a minimum cytogenetic response to therapy, one patient achieved a complete cytogenetic remission, and only 1 patient failed to demonstrate a cytogenetic response. A six-month therapy was associated with a complete clinical-and-hematological remission in 4 (80%) patients, with its duration ranging between 4 to 5 months, all patients maintaining a minimum cytogenetic response to vellferon treatment. Thus, treatment with vellferon permits obtaining not only a clinical and hematological but also a cytogenetical response to therapy in a major proportion of CML patients.

[The efficacy of polychemotherapy programs in treating multiple myeloma patients]. / Effektivnost' nekotorykh programm polikhimioterapii pri lechenii bol'nykh mnozhestvennoi mielomoi.

AIM: To compare efficiency of the programs MCVP, VCAP and ARA-COP in the treatment of multiple myeloma (MM) as regards completeness of the response, duration of the remission and toxicity. MATERIALS AND METHODS: A total of 41 MM patients entered the study (27 females, 14 males, age from 41 to 72 years, MM duration from 1 month to 8 years). 16, 10 and 15 patients were treated according to MCVP, VCAP and ARA-COP programs. RESULTS: Both in the resistant and primary patients the response was the highest to ARA-COP treatment. The remission or stabilization was achieved in 93.4% of patients. VCAP program was less effective. However, clinicohematological remission was achieved in 50% of patients. This program is rather heart toxic. MCVP program was the least effective. Survival was followed up in 16 patients (10 MCVP, 3 VCAP and 3 ARA-COP patients). The survival was 20-62, 16-36, 23.6-64.8 months for ARA-COP, VCAP and MCVP, respectively. CONCLUSION: ARA-COP program proved most effective of the three programs both in primary and drug-resistant patients. VCAP and MCVP programs are less effective but can be used in primary management of MM patients.