lymphoma

Extensive Combined Surgeries in Patients with Blood Diseases and Gastric Cancer

SURGERY IN ONCOHEMATOLOGY , , , ,

S.R. Karagyulyan, K.I. Danishyan, A.V. Grzhimolovskii, V.S. Shavlokhov, S.A. Shutov, M.A. Silaev, L.G. Kovaleva, O.M. Sorkina, A.Yu. Bulanov

Hematology Research Center of RF MH, Moscow, Russian Federation

For citation: Karagyulyan S.R., Danishyan K.I., Grzhimolovskii A.V., Shavlokhov V.S., Shutov S.A., Silaev M.A., Kovalyova L.G., Sorkina O.M., Bulanov A.Yu. Extensive Combined Surgeries in Patients with Blood Diseases and Gastric Cancer. Klin. onkogematol. 2014; 7(3): 335–42 (In Russ.).

ABSTRACT

The second tumor in patients with myelo- or lymphproliferative diseases is not rare. However, the state of patients’ hemostasis and severely enlarged spleen can become a contraindication for a surgical intervention in a general surgery unit. Four cases of a successful gastrectomy with expanded lymphodissection are presented in a 53 year old woman with subleukemic myelosis (primary myelofibrosis according to WHO classification) and giant splenomegaly (38 ´ 21 ´ 13 cm, spleen weight: 4500 g) and in a 68 year old man with diffuse large В-cell lymphoma and massive splenomegaly (22 ´ 12 ´ 8 cm, spleen weight: 2850 g). Intraoperative compensated blood loss in both cases was 3800 ml and 3740 ml, respectively. Two patients had mild hemophilia A. Specially designed transfusional and anaesthetic strategy was required in all cases because of hemostatic disorders.

Keywords: stomach cancer, surgical treatment, hemophilia, myelofibrosis, lymphoma.

Address correspondence to: max-blood@mail.ru

Accepted: May 28, 2014

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Autoimmune lymphoproliferative syndrome: literature review and case report

RARE AND COMPLEX HEMATOLOGICAL SYNDROMES , , , , , , ,

I.B. Kovynev1, T.I. Pospelova1, A.V. Mishenin2, I.N. Nechunaeva2, and R.V. Tarnovsky1

1 Novosibirsk State Medical University, Novosibirsk, Russian Federation

2 Municipal Clinical Hospital #2, Novosibirsk, Russian Federation

ABSTRACT

The article presents the literature review and the rare case of autoimmune lymphoproliferative syndrome (ALPS). The disease is associated with blocked apoptosis of immune cells caused by mutations in Fas-protein genes. The clinical course is characterized by progressive generalized lymphadenopathy with the picture of reactive hyperplasia in lymph nodes biopsy specimens and immune 1- to 3-lineage cytopenia(s). Potential of transformation into lymphoma or systemic lupus erythematosus (SLE) and current treatment approaches are discussed

Keywords: autoimmune lymphoproliferative syndrome, cytopenias, autoimmune hemolytic anemia, autoimmune neutropenia, lymphadenopathy, lymphoproliferative disease, immune thrombocytopenic purpura, lymphoma

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