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1. Male HJ, Davis MB, McGuirk JP, Abhyankar S, Aljitawi OS, Zhang D, Ganguly S: Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission. Int J Hematol; 2010 Sep;92(2):398-400
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Blastic plasmacytoid dendritic cell neoplasm should be treated with acute leukemia type induction chemotherapy and allogeneic stem cell transplantation in first remission.
  • Blastic plasmacytoid dendritic cell (BPDC) neoplasm is a rare but clinically aggressive tumor known to be derived from the precursors of plasmacytoid dendritic cells (CD123+) with a high frequency of cutaneous and bone marrow involvement.
  • Though majority of the patients initially respond to multi-agent chemotherapy, most would relapse within a year.
  • We hereby report a patient with disseminated cutaneous BPDC with marrow involvement diagnosed by typical histo-pathological and flow-cytometric findings.
  • He was subsequently treated with leukemia type induction regimen followed by allogeneic stem cell transplantation in first complete remission.
  • We recognize that BPDC with marrow involvement behaves like acute myeloid leukemia and aggressive treatment followed by stem cell transplantation may lead to long-term remission in selected cases.
  • [MeSH-major] Dendritic Cells / pathology. Hematopoietic Stem Cell Transplantation / methods. Leukemia / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Bone Marrow. Humans. Male. Middle Aged. Remission Induction. Skin Neoplasms / therapy. Transplantation Chimera. Transplantation, Homologous


2. Greer JP, Mosse CA: Natural killer-cell neoplasms. Curr Hematol Malig Rep; 2009 Oct;4(4):245-52
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  • [Title] Natural killer-cell neoplasms.
  • The natural killer (NK)-cell neoplasms are rare, representing less than 1% of non-Hodgkin lymphoma, except in Asia and Latin America, where they represent 3% to 6%.
  • NK-cell neoplasms include immature acute leukemias; a blastic NK-cell lymphoma, which is obsolete because of its plasmacytoid dendritic-cell origin; and mature NK neoplasms, comprising extranodal NK/T-cell lymphoma (ENKL), nasal-type; aggressive NK-cell leukemia; and chronic NK-cell lymphoproliferative disorders, which are often reactive.
  • Epstein-Barr virus is usually detected in tumor cells of ENKL and aggressive NK-cell leukemia.
  • The latter two mature NK neoplasms are relatively chemoresistant because of the frequent expression of P-glycoprotein.
  • Early radiation is advocated for localized nasal ENKL.
  • Stem cell transplantation is recommended for advanced disease, owing to a poor prognosis.
  • Novel agents, including chemotherapy, inhibitors of molecular pathways, and monoclonal antibodies, are under investigation.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Lymphoma / pathology. Lymphoproliferative Disorders / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cell Lineage. Cell Transformation, Neoplastic. Humans. Stem Cell Transplantation

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  • (PMID = 20425414.001).
  • [ISSN] 1558-822X
  • [Journal-full-title] Current hematologic malignancy reports
  • [ISO-abbreviation] Curr Hematol Malig Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 42
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3. Kohrt H, Advani R: Extranodal natural killer/T-cell lymphoma: current concepts in biology and treatment. Leuk Lymphoma; 2009 Nov;50(11):1773-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extranodal natural killer/T-cell lymphoma: current concepts in biology and treatment.
  • Natural killer/T-cell (NK/T) lymphomas represent a group of rare tumors of NK and NK-T cells.
  • The World Health Organization classifies NK-cell tumors into three types, extranodal NK/T-cell lymphomas (ENKL, nasal and non-nasal), NK-cell leukemias, and a blastic variant (CD4-positive, CD56-positive hematodermic neoplasms).
  • Though considerable advances have been made in our understanding of NK-cell biology, malignant transformation including the role of Epstein-Barr virus, and prognosis, the rare nature of ENKL and its heterogeneity limit the ability to standardize therapy.
  • Radiotherapy is fundamental to treatment of early-stage disease with a role for chemoradiotherapy among high-risk patients.
  • Therapeutic approaches to advanced-stage or relapsed and refractory disease, including the appropriate sequence of chemotherapy, combined modality therapy, and stem cell transplantation is not well-established.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / pathology. Lymphoma, Extranodal NK-T-Cell / therapy
  • [MeSH-minor] Combined Modality Therapy. Drug Therapy. Hematopoietic Stem Cell Transplantation. Humans. Neoplasm Staging. Prognosis. Radiotherapy. Survival Analysis

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  • (PMID = 19883307.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / T32 HL007952
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 84
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4. Northup JK, Gadre SA, Ge Y, Lockhart LH, Velagaleti GV: Do cytogenetic abnormalities precede morphologic abnormalities in a developing malignant condition? Eur J Haematol; 2007 Feb;78(2):152-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Do cytogenetic abnormalities precede morphologic abnormalities in a developing malignant condition?
  • Here, we highlight two cases in which the cytogenetic studies challenge the common practice of using hematologic and morphologic changes as key factors in malignant disease management.
  • The first case is that of a lymph node sample from a 40-yr-old non-Hodgkin's lymphoma (NHL) patient sent for determination of disease progress.
  • After two cycles of chemotherapy with fludarabine, the patient did not show any clinical response, suggesting possible progression to high-grade lymphoma.
  • The second case is of a patient with a history of human immunodeficiency virus and blastic natural killer leukemia/lymphoma.
  • Hematologic studies of ascitic fluid classified the patient as having pleural effusion lymphoma whereas bone marrow analysis showed no malignancy.
  • Bone marrow cytogenetic studies showed multiple clonal abnormalities including a t(8;14), which is commonly associated with Burkitt's lymphoma (BL).
  • To our knowledge, this is the first case wherein a morphologically normal bone marrow showed presence of clonal abnormalities consistent with BL or Pleural effusion lymphoma.
  • After two cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, the patient's general condition and ascitis improved and she was discharged.
  • These studies clearly demonstrate that genetic changes often precede morphologic changes in a developing malignant condition.
  • Therefore, the critical information needed for care of patients with malignant disorders may be incomplete or inaccurate if cytogenetic evaluation is overlooked.
  • [MeSH-major] Burkitt Lymphoma / genetics. Lymphoma, AIDS-Related / genetics. Lymphoma, Follicular / genetics. Lymphoma, Non-Hodgkin / genetics. Translocation, Genetic
  • [MeSH-minor] Adult. Antibodies, Monoclonal / administration & dosage. Antibodies, Monoclonal, Murine-Derived. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow / pathology. Chromosomes, Human, Pair 12 / ultrastructure. Chromosomes, Human, Pair 14 / genetics. Chromosomes, Human, Pair 14 / ultrastructure. Chromosomes, Human, Pair 18 / genetics. Chromosomes, Human, Pair 18 / ultrastructure. Chromosomes, Human, Pair 8 / genetics. Chromosomes, Human, Pair 8 / ultrastructure. Chromosomes, Human, X. Clone Cells / pathology. Cyclophosphamide / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Drug Resistance, Neoplasm. Female. Genes, myc. Humans. Karyotyping. Lymph Nodes / pathology. Male. Mutagenesis, Insertional. Pleural Effusion, Malignant / drug therapy. Pleural Effusion, Malignant / genetics. Pleural Effusion, Malignant / pathology. Prednisone / administration & dosage. Rituximab. Trisomy. Vidarabine / administration & dosage. Vidarabine / analogs & derivatives. Vincristine / administration & dosage

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  • (PMID = 17313561.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 4F4X42SYQ6 / Rituximab; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine; VB0R961HZT / Prednisone; CHOP protocol
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5. Oshimi K: NK cell lymphoma. Int J Hematol; 2002 Aug;76 Suppl 2:118-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] NK cell lymphoma.
  • Natural killer (NK) cells are lymphocytes with large granular lymphocyte morphology, CD3-CD56+ phenotype, non-MHC-restricted cytotoxicity, and germ-line configuration T-cell receptor genes.
  • Two types of lymphomas originating from NK cells have been described; blastic NK-cell lymphoma, and nasal-type NK-cell lymphoma.
  • Because recent reports indicate that blastic NK-cell lymphoma originates from the precursors of plasmacytoid dendritic cells, I will focus mainly on nasal-type NK-cell lymphoma, and discuss its pathogenesis, diagnostic problems, treatment strategy, and outcome.
  • Nasal-type NK-cell lymphoma develops mostly in the nasal cavity and rarely in other sites, such as the skin and intestinal tract.
  • Epstein-Barr virus (EBV) is found in lymphoma cells of almost all the patients, and is considered to be the etiologic agent.
  • Indeed, EBV easily infects NK cells in the absence of CD21 antigen, or EBV receptor, on the surface of NK cells.
  • Based on the data obtained from paraffin-embedded specimens, it is difficult to determine whether the lymphoma cells are of T-cell or NK-cell lineage, because immunohistochemical staining of cytoplasmic CD3 is positive both in T and NK cells, and CD56 is positive in a part of T cells.
  • The presence of CD5 antigen indicates T-cell lineage.
  • In advanced stages, a combination chemotherapy including L-asparaginase seems to be promising, and high-dose chemotherapy with autologous or allogeneic stem cell support is under investigation.
  • A recent report described the expression of short-length P-glycoprotein (P-gp), but not full-length P-gp in NK cells, and this mini-P-gp is unable to extrude daunorubicin.
  • Finally, I will present the results on interim analysis of 166 cases of nasal-type NK-cell lymphoma collected in Japan between 1994 and 1998.
  • [MeSH-major] Killer Cells, Natural. Lymphoma, T-Cell / pathology
  • [MeSH-minor] Combined Modality Therapy. Humans. Nose Neoplasms / diagnosis. Nose Neoplasms / etiology. Nose Neoplasms / pathology

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  • (PMID = 12430911.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 23
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6. Leitenberger JJ, Berthelot CN, Polder KD, Pro B, McLaughlin P, Jones D, Duvic M: CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate. J Am Acad Dermatol; 2008 Mar;58(3):480-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate.
  • The CD4(+) CD56(+) hematodermic/plasmacytoid dendritic cell tumor is a rare, highly aggressive, systemic neoplasm for which effective therapies have not yet been established.
  • These tumors express CD4, CD56, CD123, and T-cell leukemia/lymphoma (TCL)-1 and are clinically characterized by cutaneous involvement with spread to bone marrow and blood, and poor prognosis with current chemotherapy regimens.
  • We describe a Caucasian woman who presented with plasmacytoid dendritic cell tumor, but an absence of systemic symptoms.
  • Clinically, multiple cutaneous lesions were brown to violaceous firm nodules on the face, arms, and trunk.
  • The investigational agent, pralatrexate (30 mg/m(2)) was given weekly with vitamin B12 and folic acid and resulted in remarkable clinical response with regression of skin tumors.
  • Our observation highlights pralatrexate as a promising therapeutic option for hematodermic/plasmacytoid dendritic cell lymphoma/leukemias.
  • [MeSH-major] Aminopterin / analogs & derivatives. Antigens, CD4 / analysis. Antigens, CD56 / analysis. Dendritic Cells / pathology. Lymphoma, T-Cell / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Drug Therapy, Combination. Female. Folic Acid / therapeutic use. Humans. Killer Cells, Natural / pathology. Neoplasm Recurrence, Local. Positron-Emission Tomography. Prednisone / therapeutic use. Treatment Outcome. Vincristine / therapeutic use. Vitamin B 12 / therapeutic use

  • MedlinePlus Health Information. consumer health - Skin Cancer.
  • COS Scholar Universe. author profiles.
  • Hazardous Substances Data Bank. DOXORUBICIN .
  • Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .
  • Hazardous Substances Data Bank. PREDNISONE .
  • Hazardous Substances Data Bank. AMINOPTERIN .
  • Hazardous Substances Data Bank. VINCRISTINE .
  • Hazardous Substances Data Bank. FOLIC ACID .
  • Hazardous Substances Data Bank. CYANOCOBALAMIN .
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  • (PMID = 18280345.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA16672; United States / NCI NIH HHS / CA / K24-CA86815
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 10-propargyl-10-deazaaminopterin; 0 / Antigens, CD4; 0 / Antigens, CD56; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 935E97BOY8 / Folic Acid; JYB41CTM2Q / Aminopterin; P6YC3EG204 / Vitamin B 12; VB0R961HZT / Prednisone; CHOP protocol
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7. Chang HJ, Lee MD, Yi HG, Lim JH, Lee MH, Shin JH, Choi SJ, Moon Y, Nahm CH, Kim CS: A case of blastic plasmacytoid dendritic cell neoplasm initially mimicking cutaneous lupus erythematosus. Cancer Res Treat; 2010 Dec;42(4):239-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of blastic plasmacytoid dendritic cell neoplasm initially mimicking cutaneous lupus erythematosus.
  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease.
  • A 67-year-old man complained of skin rashes on his back and this spread to the trunk, face, arms and thighs, and he was initially diagnosed with cutaneous lupus erythematosus according to the skin biopsy.
  • Repeated skin biopsy revealed a diffuse infiltration of lymphoid cells with medium sized nuclei, positive for CD4 and CD56, negative for Epstein-Barr virus (EBV), indicating a diagnosis of BPDCN.
  • Further workups confirmed stage IVA BPDCN involving the skin, multiple lymph nodes, the peripheral blood and the bone marrow.
  • Herein we report on a rare case of BPDCN that was initially misinterpreted as cutaneous lupus erythematosus.

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  • (PMID = 21253327.001).
  • [ISSN] 2005-9256
  • [Journal-full-title] Cancer research and treatment : official journal of Korean Cancer Association
  • [ISO-abbreviation] Cancer Res Treat
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC3021744
  • [Keywords] NOTNLM ; Cutaneous lupus erythematosus / Drug therapy / Neoplasm / Plasmacytoid dendritic cells
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