The Experts below are selected from a list of 453 Experts worldwide ranked by ideXlab platform

Hervé Tilly - One of the best experts on this subject based on the ideXlab platform.

  • intense paraneoplastic neutrophilic Leukemoid Reaction related to a g csf secreting lung sarcoma
    American Journal of Hematology, 2005
    Co-Authors: Fabrice Jardin, Marc Debled, Stephane Dominique, Philippe Courville, Francoise Callonnec, G Buchonnet, Luc Thiberville, Marc Vasse, Hervé Tilly
    Abstract:

    A white blood cell count more than 50 · 10 9 /l, not related to bone marrow involvement, is termed Leukemoid Reaction. We report on the first case of an undifferentiated sarcoma of the lung associated with an intense paraneoplastic neutrophilic Leukemoid Reaction related to the production of granulocyte colony-stimulating factor (G-CSF). A radiography and a computed tomography scan of the chest revealed a well-limited voluminous and heterogeneous low-density mass of the left lung. The patient died of multiorgan failure related to uncontrolled progressive tumor growth after admission and two cycles of chemotherapy. The patient’s G-CSF serum concentration was dramatically elevated (6,538 pg/ml) compared to serum levels observed in normal controls and patients with elevated leukocytosis (31 and 387 pg/ml, respectively). The G-CSF concentration dramatically increased after the first cycle of chemotherapy and during the subsequent neutropenia, as a result of the tumor lyses as well as of disruption of the physiological negative feedback mechanism. Adjunction of the patient’s serum to CD34+ cell cultures induced a 12.3-fold increase in CD15+ cells, demonstrating the serum’s capacity to induce myeloid differentiation. Am. J. Hematol. 80:243–245, 2005. a 2005 Wiley-Liss, Inc.

Koji Kato - One of the best experts on this subject based on the ideXlab platform.

  • frequent mutations in the gata 1 gene in the transient myeloproliferative disorder of down syndrome
    Blood, 2003
    Co-Authors: Gang Xu, Masumi Nagano, Rika Kanezaki, Tetsuo Mitui, Tsutomu Toki, Tomohiko Taki, Takeshi Taketani, Yasuhide Hayashi, Kenichi Koike, Koji Kato
    Abstract:

    Transient myeloproliferative disorder (TMD) is a Leukemoid Reaction occurring occasionally in Down syndrome newborn infants. Acute megakaryocytic leukemia (AMKL) develops in approximately 20% to 30% of the cases with TMD. Recently, acquired mutations in the N-terminal activation domain of the GATA-1 gene, encoding the erythroid/megakaryocytic transcription factor GATA-1, have been reported in Down syndrome-related AMKL (DS-AMKL). To understand the multistep leukemogenesis in Down syndrome, GATA-1 mutations were investigated in patients with TMD. We show here that mutations in the GATA-1 gene were detected in 21 of 22 cases with TMD. Most of the mutations in TMD were located in the regions including exon 2 and were essentially identical to those observed in DS-AMKL. In the DS-AMKL cell line, MGS, which itself expresses only a truncated mutant of GATA-1, expression of full-length GATA-1 induced the differentiation toward the erythroid lineage. However, expression of the short form of GATA-1 did not induce erythroid differentiation. These results indicate that expression of GATA-1 with a defective N-terminal activation domain contributes to the expansion of TMD blast cells and that other genetic changes contribute to the development of AMKL in Down syndrome.

Hans Jurgen Klomp - One of the best experts on this subject based on the ideXlab platform.

  • paraneoplastic Leukemoid Reaction and rapid progression in a patient with malignant melanoma establishment of kt293 a novel g csf secreting melanoma cell line
    Cancer Biology & Therapy, 2005
    Co-Authors: Bodo Schniewind, Matthias Christgen, Axel Hauschild, Roland Kurdow, Holger Kalthoff, Hans Jurgen Klomp
    Abstract:

    Paraneoplastic Leukemoid Reaction (PLR) is a rare condition of leucocytosis in cancer patients. Here we report the rapid progression of a patient suffering from a metastasized malignant melanoma and PLR. The patient's white blood cell count exceeded 200,000 cells per mul and the serum level of Granulocyte Colony-Stimulating Factor (G-CSF) was elevated up to 780 pg/mul. A Tc-m99-labeled anti-NCA90/95 based granulocyte scan demonstrated reactive bone marrow expansion, splenomegaly and granulocyte infiltration into the tumor. KT293, a S100, gp100 and CD68 positive melanoma cell line derived from an axillary metastasis, produced large amounts of G-CSF in vitro and induced rapidly growing tumors and PLR after subcutaneous inoculation in SCID mice. In contrast to G-CSF-secreting cancer cells of other tissue origin, G-CSF-neutralizing antibodies failed to inhibit the growth of KT293 cells. In addition, KT293 cells did not express G-CSF-receptor. These observations suggest that paracrine effects of G-CSF-secretion and PLR might promote an aggressive melanoma phenotype, as seen in this patient.

Yoshihiko Maehara - One of the best experts on this subject based on the ideXlab platform.

  • gastric adenosquamous carcinoma producing granulocyte colony stimulating factor
    Gastric Cancer, 2005
    Co-Authors: Kazuya Endo, Shunji Kohnoe, Takeshi Okamura, Masaru Haraguchi, Eisuke Adachi, Yasushi Toh, Hideo Baba, Yoshihiko Maehara
    Abstract:

    We report a case of adenosquamous carcinoma of the stomach that produced granulocyte-colony stimulating factor (G-CSF). The patient, who had an admission diagnosis of advanced gastric cancer, had marked leukocytosis without evidence of infection. After leukemia and metastatic Leukemoid Reaction were excluded by bone marrow examination, a G-CSF-producing cancer was suspected as the cause of the abnormally elevated serum G-CSF level. The resected stomach tumor was histologically diagnosed as adenosquamous carcinoma; positive expression of G-CSF by tumor cells was shown with immunohistochemical detection, which confirmed the preoperative diagnosis. Recurrent disease in the liver and lymph nodes, accompanied by leukocytosis and re-elevation of serum G-CSF, developed just 3 months after the curative gastrectomy and adjuvant chemotherapy. All of the recurrent disease was resected, restoring normal levels of serum G-CSF. The patient survived for almost 2 years after the initial surgery with extensive chemotherapy, including weekly treatment with paclitaxel, before finally succumbing to liver failure secondary to extensive liver metastasis.

Marianne Frieri - One of the best experts on this subject based on the ideXlab platform.

  • inflammatory malignant fibrous histiocytoma associated with Leukemoid Reaction or leukocytosis a comprehensive review
    International Scholarly Research Notices, 2012
    Co-Authors: Jorge Hurtadocordovi, Prajwol Pathak, Boris Avezbakiyev, Marianne Frieri
    Abstract:

    Inflammatory malignant fibrous histiocytoma (IMFH) associated with Leukemoid Reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, Leukemoid Reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.

  • Review Article Inflammatory Malignant Fibrous Histiocytoma Associated with Leukemoid Reaction or Leukocytosis: A Comprehensive Review
    2012
    Co-Authors: Jorge Hurtado-cordovi, Prajwol Pathak, Boris Avezbakiyev, Marianne Frieri
    Abstract:

    Copyright © 2012 Jorge Hurtado-Cordovi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Inflammatory malignant fibrous histiocytoma (IMFH) associated with Leukemoid Reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant’s response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, Leukemoid Reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 1

  • Cutaneous Inflammatory Malignant Fibrous Histiocytoma Presenting with a Leukemoid Reaction: A Case Report and Review of the Literature
    Hindawi Limited, 2012
    Co-Authors: Jorge Hurtado-cordovi, Boris Avezbakiyev, Marianne Frieri, Lester Freedman, Wondwoosen Gebre
    Abstract:

    Malignant fibrous histiocytoma (MFH) is the most common sarcoma found in adults. We discuss a case of inflammatory MFH of dermal/epidermal origin presenting with a severe Leukemoid Reaction (LR). A 60 years old white male presented to hematology/oncology clinic complaining of mild shortness of breath on exertion. Past medical history was remarkable for removal of a left upper extremity necrotic mass 4.4 × 3 × 3 cm. Microscopy of the specimen showed clear surgical margin, and tumor cells restricted to the dermis without lymphovascular invasion. Immunohistochemestry was positive for CD 68 and CD 99. Chest x-ray was negative for metastatic disease. White blood cell count was 109.4 k/mm3 with 24 k/mm3 band neutrophils, and absolute neutrophil count of 69 k/mm3. CT scan of the thorax revealed numerous bilateral pulmonary nodules suspicious for metastasis. Based on these findings patient was diagnosed with metastatic cutaneous IMFH associated with a LR. Following review of medical literature, this appears to be the first reported case of inflammatory cutaneous MFH associated with LR. This histological variant is rare, and carries a poor prognosis. Thus, we would like to emphasize the need for investigating alternative therapies capable of improving the survival of these patients