Sezary syndrome in a patient with hairy cell leukemia in remission

Crump, Michael; Sutton, David; Pantalony, Dominic

doi:10.1002/1097-0142(19910815)68:4<829::aid-cncr2820680427>3.0.co;2-1

Cited by 7 publications

(3 citation statements)

References 27 publications

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“…The related nature of HCL, ATL, and CTCL is further underscored by the finding that HCL and ATL as well as HCL and CTCL can coexist in the same patient. [77][78][79][80][81] In addition, HCL, ATL, and CTCL share the characteristic of being amenable to treatment with pentostatin or chlorodeoxyadenosine. [82][83][84][85][86] The molecular and pathogenic similarities of HCL, ATL, and CTCL tend to suggest these malignancies share common origins.…”

Section: Discussionmentioning

confidence: 99%

CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras andjunD

Nicolaou

Teodoridis

Park

et al. 2003

Blood

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IntroductionHairy cell leukemia (HCL) or leukemic reticuloendotheliosis represents approximately 2% of adult leukemias and is characterized by pancytopenia, hepatomegaly, splenomegaly, leukocytosis, and neoplastic mononuclear cells in the peripheral blood, bone marrow, liver, and spleen. 1 The name of the disease is derived from the presence of broad-based undulating ruffles on the surface of the leukemic cells that appear under the phase contrast microscope as cytoplasmic projections or "hairs." These cells can be derived both from B and T lymphocytes as demonstrated by their expression of B-or T-cell-specific antigens and are characterized biochemically by their abnormal expression of the integrin heterodimer CD11c/ CD18. [2][3][4] Under normal circumstances the gene encoding the CD18 component of this marker is transcribed both in lymphocytes and myeloid cells, whereas the gene encoding the CD11c component is transcribed primarily in cells of the myeloid lineage. 5 The CD11c/ CD18 heterodimer is, therefore, normally largely restricted in its expression to the surface of myeloid cells dictated by the myeloidspecific transcription of the CD11c gene. In hairy cell leukemia the CD11c/CD18 heterodimer is present on not only myeloid cells but also on the neoplastic lymphocytes. Diagnostic of this disease is, therefore, the abnormal regulation of CD11c gene transcription. Consequently, elucidation of the molecular causes of this abnormal regulation are likely to result in insights into the molecular basis of hairy cell leukemia. Using this rationale we isolated the human CD11c gene and identified the cis-acting elements that control its transcription. The most important of these elements interacts with activator protein-1 (AP-1) encoded by the jun and fos families of proto-oncogenes. In hairy cells an AP-1 complex containing JunD exhibits a constitutive pattern of expression, whereas in other cell types it is functionally expressed only upon induction with phorbol ester. The use of dominant-negative mutants in transfection assays demonstrated that both AP-1 and its upstream activator Ras are necessary for CD11c expression in hairy cells. In addition, exogenous expression of a dominant-positive mutant of Ras was able to activate the CD11c promoter in nonhairy cells to a level equivalent to that seen in hairy cells. Taken together, these results indicate that activation of the proto-oncogenes junD and ras underlie the abnormal expression of the CD11c gene characteristic of HCL. Materials and methods Cell cultureThe cell lines HeLa, IM-9, Mo, and U937 were obtained from the American Type Culture Collection (Manassas, VA) and grown according to their specifications. The promegakaryocytic cell line MEG-01 6 was kindly provided by Dr W. S. May (John Hopkins Oncology Center, Baltimore, MD) through permission of Dr H. Saito (Nagoya University School of Medicine, Nagoya, Japan). MEG-01 were cultured in RPMI 1640 medium supplemented with glutamine, 20% fetal calf serum (FCS), aqueous penicillin G (100 U/mL), and streptomycin ...

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Section: Discussionmentioning

confidence: 99%

CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras andjunD

Nicolaou

Teodoridis

Park

et al. 2003

Blood

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“…SS has been associated with hemophagocytic syndrome-induced anemia, normocytic anemia secondary to bone marrow infiltration, and pancytopenia [4][5][6]. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has been observed as dose-dependent toxicity of pentostatin treatment for SS [7].…”

Section: Discussionmentioning

confidence: 99%

Fatal Microangiopathic Hemolytic Anemia Due to Sézary Syndrome

Robertson¹,

Jafry²,

Soma³

et al. 2021

Cureus

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Sézary syndrome (SS) is a form of cutaneous T-cell lymphoma (CTCL), demonstrating leukemic involvement of malignant T-cells. Known systemic sequelae of SS include hemophagocytic syndrome-induced anemia, normocytic anemia secondary to bone marrow infiltration, and pancytopenia. We report a patient with SS, initially demonstrating widespread morbilliform eruption, who presented with malignancy-related microangiopathic hemolytic anemia (MAHA). Our findings represent a novel presentation of SS that will inform the differential diagnosis and treatment of future SS patients presenting with anemia and thrombocytopenia.

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“…55 associated with malignant tumors is extremely rare. Up to the present, it has been reported to be associated with colon cancer (20), double cancer of the esophagus and lung (21), hepatoma (22), and hair cell leukemia (23). The mechanism of these associations is presumed to be a proliferation of neoplastic cells due to some changes in organization of tumor cell elimination, which is caused by decreasing immune function after leukemia degeneration of the helper T cells in 55 (22).…”

Section: Report Of a Casementioning

confidence: 99%

Leser‐Trélat Sign Associated with Sézary Syndrome

Ikari

Ohkura

Morita

et al. 1995

The Journal of Dermatology

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A 74-year-old Japanese male had developed generalized erythroderma and rapid growth of multiple verrucous lesions over the entire surface of his face, trunk, and extremities three months before he was seen. Histologically seborrheic keratoses were revealed. Laboratory examinations showed peripheral leukocytosis with atypical lymphocytes and high levels of IgE and IgG. On the basis of these clinical and histopathologic findings, we diagnosed the patient as having Leser-Trélat sign associated with Sézary syndrome. The erythroderma subsided after administration of oral predonisone, and no new formations of seborrheic keratosis were observed. However, because of subsequent aggravation of the generalized erythroderma, we administered chemotherapy. Six months after the initial examination, lung cancer was found, and the patient subsequently died of respiratory and renal failure.

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Sezary syndrome in a patient with hairy cell leukemia in remission

Cited by 7 publications

References 27 publications

CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras andjunD

CD11c gene expression in hairy cell leukemia is dependent upon activation of the proto-oncogenes ras andjunD

Fatal Microangiopathic Hemolytic Anemia Due to Sézary Syndrome

Leser‐Trélat Sign Associated with Sézary Syndrome

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