treatment

Interdigitating dendritic cell sarcoma: case report and literature review

RARE HEMATOLOGICAL TUMORS AND SYNDROMES , ,

P.A. Zejnalova1, А.I. Pavlovskaya1, T.T. Valiev2, and N.V. Kokosadze1

1 Clinical Oncology Research Institute, N.N. Blokhin Cancer Research Center, RAMS, Moscow, Russian Federation

2 Pediatric Oncology and Hematology Research Institute, N.N. Blokhin Cancer Research Center, RAMS, Moscow, Russian Federation

ABSTRACT

We present the literature data and the authors’ observation of interdigitating dendritic cell sarcoma (IDCS), a very rare tumor consisting of histiocytic and dendritic cells. Due to variability in clinical presentation with nodal and/or extranodal involvement and immunomorphological characteristics of this sarcoma, differential diagnosis with non-Hodgkin’s lymphomas, melanoma, follicular dendritic cell sarcoma, and Langerhans cell histiocytosis is necessary. Here, we describe a clinical case of IDCS with cervical lymph node involvement and successful treatment with a CHOP program and subsequent irradiation. No generally accepted treatment strategy exists so far. Currently used methods include surgery, irradiation, and chemotherapy, but treatment outcomes need to be optimized.

Keywords: Interdigitating dendritic cell sarcoma, diagnosis, treatment.

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 REFERENCES

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Mantle cell lymphoma: program therapy for untreated patients under 65 years

LYMPHOID TUMORS , , ,

V.I. Vorobyev1, S.K. Kravchenko1, E.G. Gemdjian1, Yu. Yu. Lorie 2, A.U. Magomedova1, A.L. Melikyan1, J.K. Mangasarova1, D.S. Mar’yin1, E.I. Dubrovin1, T.N. Obukhova1, S.A. Makhinya, V.A. Zherebtsova3, M.A. Vernyuk4, N.G. Tyurina4, and V.G. Savchenko1

1 Hematology Research Center, RF Ministry of Health, Moscow, Russian Federation

2 Moscow Oncology Clinic #3, Russian Federation

3 Central Clinical Hospital with Polyclinic, RF Presidential Executive Office, Moscow, Russian Federation

4 P.A. Hertzen Moscow Oncology Research Institute, Moscow, Russian Federation

ABSTRACT

Background: Mantle cell lymphoma (MCL) is aggressive B-cell neoplasm which is diagnosed predominantly among older men. The use of high-dose Ara-C (12 g/m2 per course), autoSCT, and rituximab at all stages of therapy is the most effective approach but it is feasible only in patients under 60–65 years. High efficacy of gemcitabine and oxaliplatin-based regimens and irinotecan in relapsed or refractory MCL justifies their use in first-line therapy.

Objective: Assessment of toxicity and efficacy of R-DA-EPOCH/R-GIDIOX- and R-DA-EPOCH/R-HD-Met-Ara-C-regimens in primary MCL patients selected for autoSCT.

Patients and Methods: Since May 2008, 41 untreated MCL pts (median age: 54 years [29–64], M/F: 73%/27%, MIPIb: 29.3% low, 36.6% intermediate, 34.1% high risk) have been enrolled. After first R-EPOCH course (W. Wilson, 2003) completed, the patients were stratified according to toxicity emerged into 2 therapeutic groups: R-DA-EPOCH/R-HD-Met-AraC or R-DA-EPOCH/R-GIDIOX. In absence of grade 4 hematological toxicity for more than 3 days, serious infectious complications, or signs of renal failure, the pts received the R-HD-Met-Ara-C (R, 375 mg/m2 on Day 0; methotrexate, 1000 mg/m2 for 24 hours, Day 1; cytarabine, 3000 mg/m2 q 12 hrs on Days 2–3) regimen. When any of the above complications was present, the pts received the R-GIDIOX (R, 375 mg/m2 on Day 0; gemcitabine, 800 mg/m2 on Days 1 and 4; oxaliplatin, 120 mg/m2 on Day 2; irinotecan, 100 mg/m2 on Day 3; dexamethasone, 10 mg/m2 IV on Days 1–5; ifosfamide, 1000 mg/m2 on Days 1–5) regimen. Then, these regimens were reversed into either R-DA-EPOCH/R-HD-Met-Ara-C or R-DA-EPOCH/R-GIDIOX. Depending on the time until the complete response was achieved, pts received 6 to 8 therapeutic courses and autoSCT (BEAM-R) with in vivo purging using rituximab. Pts with residual tumor after autoSCT underwent local irradiation. R-maintenance was performed every 3 months for 3 years. Since Nov. 2011, all pts had received intrathecal CNS prophylaxis (including the patients who had undergone autoSCT during the year preceding Nov. 2011). The protocol was approved by the local ethics committee. Pts were analyzed using the intention-to-treat model. Toxicity assessment was performed for 124 R-DA-EPOCH, 87 R-HD-Met-Ara-C, and 51 R-GIDIOX courses.

Results: The median follow-up was 22 months (range 4–60). By April 2013, 35 patients had undergone autoSCT: 21 and 14 from R-HD-Met-Ara-C- and R-GIDIOX arm, respectively. One patient died from acute renal failure and septic shock at the induction stage after first HD-Met-AraC course. R-maintenance therapy was completed in 5 patients. In all patients who had received R-HD-Met-Ara-C, CR was achieved. In the R-GIDIOX arm, OR rate was 93%: 12 CR, 2 PR, and 1 case of disease progression after 5 courses. The most common non-hematological R-GIDIOX toxicity was related to the liver with elevated aminotransferases up to Grades 1–2 and 3–4 in 64.7% and 7.8% of cases, respectively, with no clinical manifestations. The sources of stem cells was PB in 27 out of 31 patients, and in 4 cases of harvest failure after 3 R-GIDIOX and 1 HD-Met-AraC BM was used. Hematological toxicity of R-GIDIOX course included grade 4 leukopenia in 74.5% (medium duration: 5 days, range: 1–13) and grade 4 thrombocytopenia in 39.2%. The estimated 5-years OS for the R-GIDIOX and R-HD-Met-AraC groups was 93 ± 7% and 79 ± 12%, respectively. The estimated 5-years EFS for the R-GIDIOX and R-HD-Met-AraC groups was 59 ± 19% and 74 ± 12%, respectively.

Conclusions: The HD-Met-Ara-C regimen is highly toxic, and it can be used only in 2/3 of patients under 65 years. The R-GIDIOX regimen is less toxic than HD-Met-Ara-C and equally effective with regard to the response induction and mobilizing necessary amount of autologous stem cells, so it can be recommended for the patients in whom Ara-C and methotrexate in high doses carry the high risk of life-threatening consequences.

Keywords: Mantle cell lymphoma, treatment, autoSCT, maintenance therapy.

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Hodgkin’s lymphoma and a “new old” bendamustine

DIAGNOSIS AND TREATMENT OF LYMPHOID TUMORS , , ,

S.S. Shklyaev, and V.V. Pavlov

Medical Radiological Research Center, RF Ministry of Health, Obninsk, Russian Federation

ABSTRACT

Hodgkin’s lymphoma is a malignant tumor that eventually turned from a fatal incurable to successfully curable disease after primary treatment in the vast majority of cases. However, the prognosis for patients with refractory and relapsed disease is not infrequently dismal and life-threatening, especially if the tumor continues progressing after high-dose chemotherapy with autologous stem cell transplantation or, in some instances, even after allogeneic stem cell grafting. Bendamustine is a “new old” cytostatic agent that can be effectively applied for treating this group of patients. Our literature review highlights a variety of relevant options in treatment of Hodgkin’s lymphoma using bendamustine.

Keywords: Hodgkin’s lymphoma, refractory and relapsed disease, treatment, bendamustine.

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Childhood testicular diffuse large B-cell lymphoma

DIAGNOSIS AND TREATMENT OF LYMPHOID TUMORS , , ,

T.T. Valiev1, A.M. Kovrigina2, I.N. Serebryakova3, E.V. Mikhailova1, A.V. Popa1, G.L. Mentkevich1

1-Paediatric Research Institute of FSBI «Named after N.N. Blokhin Russian Cancer Research Center» RAMS, Moscow.

2-FSBI «Haematology Research Center of The Ministry of Health of the Russian Federation», Moscow, Russian Federation

3-Clinical Oncology Research Institute of FSBI «Named after N.N. Blokhin Russian Cancer Research Center» RAMS, Moscow, Russian Federation

ABSTRACT

Non-Hodgkin’s lymphomas with primary testis involvement is a rare heterogeneous group of lymphoid tumours. In adults testicular lymphomas (TL) are characterized by aggressive clinical progression, frequent relapses and metastases to CNS, and poor response to standard therapy. According to literature data, pediatric TL presented as clinical cases. In the article Diffuse large B-cell TL in 10-year old boy presented. Authors discuss in details successful intensive chemotherapy treatment.

Keywords: testicular lymphoma, diagnosis, treatment, children

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REFERENCES

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Follicular lymphoma: current trends and my choice

MY CHOISE ,

G.S. Tumyan

FSBI «N.N. Blokhin Russian Cancer Research Center» RAMS, Moscow, Russian Federation

ABSTRACT

In this first publication in the series «My Choice», we summarize recent data on the diagnosis and treatment of follicular lymphoma and suggest an algorithm of diagnostic and therapeutic measures, which allows a hematologist to individualize treatment for untreated and relapse patients. We discuss determination of «tumor burden» criteria and disease transformation and review variants of follicular lymphoma.

Keywords: follicular lymphoma, treatment.

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REFERENCES

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