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    A Case of Radiation Recall Dermatitis of Scalp in Acute Lymphoblastic Leukemia After Prophylactic Cranial Radiotherapy.
    Cureus 2017 Sep 10;9(9):e1671. Epub 2017 Sep 10.
    Department of Medical Oncology, RCC, Jipmer, Pondicherry, India.
    The radiation recall dermatitis (RRD) phenomenon is defined as the "recalling" of the skin following the administration of drugs; this induces a response or flare-like reaction over the skin that is exposed to radiation. In this case report, a young female developed RRD on Day 18 after the completion of cranial radiotherapy, that is, four days after the restart of the chemotherapy with doxorubicin. It is a self-limiting condition with supportive care as the treatment. Read More

    Ichthyosiform Pityriasis Rubra Pilaris-Like Eruption Secondary to Ponatinib Therapy: Case Report and Literature Review.
    Drug Saf Case Rep 2017 Nov 14;4(1):19. Epub 2017 Nov 14.
    Department of Dermatology and Cutaneous Surgery, Jackson Memorial Hospital, University of Miami Miller School of Medicine, 1475 NW 12th Avenue, Miami, FL, 33136, USA.
    Tyrosine kinase inhibitors have revolutionized the chemotherapy arena as targeted therapies for a multitude of malignancies. They are more selective than conventional chemotherapy, and often elicit fewer systemic adverse events, however toxicities still exist. Cutaneous toxicities are common and their management presents a novel challenge to physicians and patients. Read More

    Suspected cancer diagnoses made by general practitioners in a population with subsequently confirmed cancer diagnoses in Germany: a retrospective study of 31,628 patients.
    Oncotarget 2017 Oct 14;8(48):84540-84545. Epub 2017 Sep 14.
    Faculty of Medicine, University of Paris 5, Paris, France.
    The goal of the present study was to analyze the prevalence and risk factors of suspected cancer diagnoses made by general practitioners (GP) in a population with subsequently confirmed cancer diagnoses in Germany. This study included patients aged 18 years or older who received an initial documentation of a confirmed cancer diagnosis from 1,262 German GP between January and December 2016 (index date). The main outcome measure of the study was the rate of suspected cancer diagnoses made by GP within one year prior to the index date. Read More

    Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.
    Clin Imaging 2017 Oct 31;49:17-36. Epub 2017 Oct 31.
    Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:
    Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

    Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

    Site of childhood cancer care in the Netherlands.
    Eur J Cancer 2017 Nov 3;87:38-46. Epub 2017 Nov 3.
    Princess Máxima Center for Pediatric Oncology, Uppsalalaan 8, Utrecht 3584 CT, The Netherlands.
    Background: Due to the complexity of diagnosis and treatment, care for children and young adolescents with cancer preferably occurs in specialised paediatric oncology centres with potentially better cure rates and minimal late effects. This study assessed where children with cancer in the Netherlands were treated since 2004.

    Methods: All patients aged under 18 diagnosed with cancer between 2004 and 2013 were selected from the Netherlands Cancer Registry (NCR) and linked with the Dutch Childhood Oncology Group (DCOG) database. Read More

    Eruption of lymphocyte recovery with atypical lymphocytes mimicking a primary cutaneous T-cell lymphoma: a series of 12 patients.
    Hum Pathol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Pathology and INSERM U955 team 9, AP-HP, Henri-Mondor hospital, 51 avenue du Maréchal Lattre de Tassigny, 94220 Créteil, France. Electronic address:
    Eruption of lymphocyte recovery (ELR) may occur during bone marrow aplasia after chemotherapies. We reviewed the clinical and pathologic features of 12 patients (male/female: 7/5, median age: 61years) with an atypical ELR histologically mimicking a primary cutaneous T-cell lymphoma (CTCL) such as Sézary Syndrome or CD30+ T-cell lymphoproliferative disorder (LPD). All the patients displayed an erythematous maculopapular eruption on the trunk and the limbs, associated with fever. Read More

    Antimicrobial and Anti-Proliferative Effects of Skin Mucus Derived from Dasyatis pastinaca (Linnaeus, 1758).
    Mar Drugs 2017 Nov 1;15(11). Epub 2017 Nov 1.
    Department of Biomedical and Biotechnological Sciences, University of Catania, Catania 95124, Italy.
    Resistance to chemotherapy occurs in various diseases (i.e., cancer and infection), and for this reason, both are very difficult to treat. Read More

    In-gap discounts in Medicare Part D and specialty drug use.
    Am J Manag Care 2017 Sep;23(9):553-559
    Department of Health Policy and Administration, College of Health and Human Development, The Pennsylvania State University, 601E Ford Building, University Park, PA 16801. E-mail:
    Objectives: Specialty drugs can bring significant benefits to patients, but they can be expensive. Medicare Part D plans charge relatively high cost-sharing costs for specialty drugs. A provision in the Affordable Care Act reduced cost sharing in the Part D coverage gap phase in an attempt to mitigate the financial burden of beneficiaries with high drug spending. Read More

    Analysis of pediatric dermatology inpatient consultations in a pediatric teaching hospital.
    Arch Argent Pediatr 2017 Dec;115(6):e377-e384
    Ataturk Research and Training Hospital, Department of Dermatology, Izmir, Turkey.
    Introduction: Although skin diseases are associated with low rate of hospitalization, dermatological manifestations are frequent in hospitalized patients. The aim of the study was to describe the inpatient dermatological consultations in a pediatric teaching hospital.

    Population And Methods: Recorded data from inpatient pediatric dermatology consultation requests on a total of 539 consecutive inpatients (aged 0-18 years) from January 2004 to April 2010 were analyzed for consult diagnosis, dermatological disease group, primary diagnosis, requesting department, diagnostic pattern, treatment modality and referral to another department. Read More

    Eosinophilic dermatosis of hematologic malignancy mimicking varicella zoster infection: report in a woman with chronic lymphocytic leukemia and review of the literature.
    Dermatol Pract Concept 2017 Jul 31;7(3):6-15. Epub 2017 Jul 31.
    Department of Dermatology, University of California San Diego, La Jolla, CA, USA.
    Eosinophilic dermatosis of hematologic malignancy is a rare papulovesicular eruption that presents in patients with hematoproliferative disorders, particularly chronic lymphocytic leukemia. A 59-year-old woman with chronic lymphocytic leukemia who developed eosinophilic dermatosis of hematologic malignancy mimicking varicella zoster infection is described. PubMed database was searched with the key words: chronic, dermatosis, eosinophilic, hematologic, infection, leukemia, lymphocytic, malignancy, varicella, zoster. Read More

    A Case of Rituximab-Induced Necrotizing Fasciitis and a Review of the Literature.
    Case Rep Hematol 2017 26;2017:6971027. Epub 2017 Sep 26.
    Department of Medicine, Reading Hospital, West Reading, PA, USA.
    Necrotizing fasciitis is a fulminant soft tissue infection characterized by rapid progression and high mortality. Rituximab is a generally well-tolerated immunosuppresive medication used for B-cell malignancies and some rheumatological disorders. We report a case of a 69-year-old male with chronic lymphocytic leukemia who suffered necrotizing fasciitis of his left lower extremity secondary to Clostridium septicum 7 weeks after treatment with rituximab. Read More

    Successful eltrombopag treatment of severe refractory thrombocytopenia in chronic myelomonocytic leukemia: Two cases reports: A CARE-compliant article.
    Medicine (Baltimore) 2017 Oct;96(43):e8337
    aDepartment of Hematology bDepartment of Pharmacology, China-Japan Friendship Hospital, Beijing, China.
    Rationale: Thrombocytopenia in chronic myelomonocytic leukemia (CMML) is usually attributed to impaired marrow production resulting from cytotoxic drug use or CMML itself ("CMML-induced thrombocytopenia"). In very rare cases, immune thrombocytopenia (ITP) can be a complication of CMML ("CMML-associated ITP"). However, treatment of severe thrombocytopenia in patients with CMML is still a challenge. Read More

    Regional incidences of adult T-cell leukemia/lymphoma with cutaneous involvement in Japan.
    J Dermatol 2017 Oct 25. Epub 2017 Oct 25.
    Departments of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
    Between 2008 and 2015, 462 newly-diagnosed adult T-cell leukemia/lymphoma (ATLL) patients with cutaneous involvement were found from the nationwide registry for Japanese patients with cutaneous lymphoma, of which 391 were selected for the study. They ranged in age from 28 to 93 years (median, 69 years), and included 215 men and 176 women (male : female ratio = 1.2). Read More

    Direct Analysis of Biofluids by Mass Spectrometry with Microfluidic Voltage-Assisted Liquid Desorption Electrospray Ionization.
    Anal Chem 2017 Nov 3. Epub 2017 Nov 3.
    Department of Chemistry and Biochemistry, Jackson State University , Jackson, Mississippi 39217, United States.
    Signal suppression by sample matrix in direct electrospray ionization-mass spectrometric (ESI-MS) analysis hampers its clinical and biomedical applications. We report herein the development of a microfluidic voltage-assisted liquid desorption electrospray ionization (VAL-DESI) source to overcome this limitation. Liquid DESI is achieved for the first time in a microfluidic format. Read More

    Merkel Cell Polyomavirus: A New DNA Virus Associated with Human Cancer.
    Adv Exp Med Biol 2017 ;1018:35-56
    Department of Microbiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
    Merkel cell polyomavirus (MCPyV or MCV) is a novel human polyomavirus that has been discovered in Merkel cell carcinoma (MCC), a highly aggressive skin cancer. MCPyV infection is widespread in the general population. MCPyV-associated MCC is one of the most aggressive skin cancers, killing more patients than other well-known cancers such as cutaneous T-cell lymphoma and chronic myelogenous leukemia (CML). Read More

    Anti-Platelet-Derived Growth Factor Receptor Alpha Chain Antibodies Predict for Response to Nilotinib in Steroid-Refractory or -Dependent Chronic Graft-versus-Host Disease.
    Biol Blood Marrow Transplant 2017 Oct 16. Epub 2017 Oct 16.
    Medicine/BMT Division, Stanford University School of Medicine, Stanford, California. Electronic address:
    Imatinib has clinical activity in chronic graft-versus-host disease (cGVHD), a significant complication of allogeneic hematopoietic cell transplant. Nilotinib is a tyrosine kinase inhibitor that targets the same receptors as imatinib but with different affinities. We tested the hypothesis that nilotinib is safe and has clinical activity in cGVHD. Read More

    Mast cell degranulation and calcium influx are inhibited by an Echinacea purpurea extract and the alkylamide dodeca-2E,4E-dienoic acid isobutylamide.
    J Ethnopharmacol 2018 Feb 14;212:166-174. Epub 2017 Oct 14.
    Department of Biological Sciences, North Carolina State University, Raleigh, NC 27695, United States; Comparative Medicine Institute, North Carolina State University, Raleigh, NC 27695, United States. Electronic address:
    Ethnopharmacological Relevance: Native Americans used plants from the genus Echinacea to treat a variety of different inflammatory conditions including swollen gums, sore throats, skin inflammation, and gastrointestinal disorders. Today, various Echinacea spp. preparations are used primarily to treat upper respiratory infections. Read More

    A possible implication of reduced levels of LIF, LIFR, and gp130 in vasculopathy related to systemic sclerosis.
    Arch Dermatol Res 2017 Dec 16;309(10):833-842. Epub 2017 Oct 16.
    Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
    Leukemia inhibitory factor (LIF) is a member of IL-6 family, which serves as a potent chemoattractant for neutrophils as well as a potent angiostatic factor. LIF has been implicated in various autoimmune inflammatory diseases, but its role still remains elusive in systemic sclerosis (SSc). Therefore, we investigated the potential role of LIF in the development of SSc by evaluating the clinical correlation of serum LIF levels, the expression of LIF and its receptors in skin samples, and in vitro experiments with human dermal microvascular endothelial cells. Read More

    Role of Mast Cells in clearance of Leishmania through extracellular trap formation.
    Sci Rep 2017 Oct 16;7(1):13240. Epub 2017 Oct 16.
    Cellular and Molecular Immunology Laboratory, School of Life Sciences, Jawaharlal Nehru University, New Delhi, 110067, India.
    Mast Cells (MCs) are one of the first immune cells encountered by invading pathogens. Their presence in large numbers in the superficial dermis, where Leishmania is encountered, suggests that they may play a critical role in immune responses to Leishmania. In this study the interactions of Leishmania donovani, the causative agent of visceral Leishmaniasis, and Leishmania tropica, the causative agent of cutaneous Leishmaniasis with MCs were studied. Read More

    [Study on the disease burden of Chinese adolescent in 2015].
    Zhonghua Yu Fang Yi Xue Za Zhi 2017 Oct;51(10):910-914
    Peking University Health Science Centre. Institute of Child and Adolescent Health of Peking University, Beijing 100191, China.
    Objective: To discuss the main causes and risk factors of disability and death among current Chinese adolescents. Methods: Subnational data of China from Global Burden of Disease Study 2015 (GBD 2015) was used to rank the causes and risk factors leading to death and disability adjusted life years (DALY) in Chinese adolescents aged between 10 and 19 years old, and thereby to analyze the main cauese and risk factors of death and DALY among Chinese adolescents in different genders. Results: In 2015, among Chinese adolescents aged 10-19 years old, the total DALY was 13 million 490 thousand years, and the total number of deaths was 63 258 cases. Read More

    Donor-lymphocyte infusion following haploidentical hematopoietic cell transplantation with peripheral blood stem cell grafts and PTCy.
    Bone Marrow Transplant 2017 Oct 16. Epub 2017 Oct 16.
    Bone Marrow Transplantation and Leukemia Program, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
    Donor-lymphocyte infusion (DLI) for relapse following haploidentical hematopoietic cell transplantation (haploHCT) with post-transplant cyclophosphamide (PTCy) has been described in recipients of bone marrow grafts, but not recipients of G-CSF mobilized peripheral blood (PB) grafts. We retrospectively identified patients who underwent DLI following PB-haploHCT with PTCy for relapse, or loss of chimerism (LOC). Twelve patients (57%) received DLI for hematologic relapse/persistent disease, seven (33%) for extramedullary relapse and two (10%) for LOC. Read More

    Chronic lymphocytic leukaemia and secondary skin malignancies.
    Br J Nurs 2017 Oct;26(18):1026-1032
    Nurse Tutor, Centre for Learning and Development at Tallaght Hospital, Dublin 24, Ireland.
    Chronic lymphocytic leukaemia (CLL) is a non-Hodgkin lymphoma commonly occurring in older adults. Many people with CLL can go for years without requiring treatment. However, they have an increased risk of secondary cancers, specifically melanoma and non-melanoma skin cancers. Read More

    Pachyderma in Primary Cutaneous NK and T-Cell Lymphoma and Leukemia Cutis.
    Case Rep Dermatol 2017 Sep-Dec;9(3):151-157. Epub 2017 Sep 11.
    Department of Dermatology, Liège University Hospital, Liège, Belgium.
    Background: Pachyderma is defined as severely thickened skin with deep folds and is occasionally observed with primary cutaneous NK and T-cell lymphoma (pCNKTCL), primary cutaneous B-cell lymphoma (pCBCL), and leukemia cutis (LC).

    Aim: To describe the clinical, histological, and therapeutic particularities of a series of pCNKTCL, pCBCL, and LC patients with pachyderma.

    Results: In a series of pCNKTCL (n = 70), pCBCL (n = 12), and LC (n = 2) patients followed up during 9 years, 6 cases of pachyderma were observed. Read More

    Outcomes of Newly Diagnosed Chronic Phase Chronic Myeloid Leukemia Following an Elective Switch From Second-Generation Tyrosine Kinase Inhibitor to Imatinib.
    Clin Lymphoma Myeloma Leuk 2017 Sep 19. Epub 2017 Sep 19.
    Department of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, GA.
    The second-generation tyrosine kinase inhibitors (TKIs) (2G-TKIs) dasatinib (DAS) and nilotinib (NIL) yield faster responses in newly diagnosed chronic phase (CP) chronic myeloid leukemia (CML) as compared with imatinib (IM); however, long-term safety of these agents is a growing concern. We identified 20 patients with CP-CML diagnosed between August 2013 and October 2016 who initiated 2G-TKIs and were then switched after optimal response at 3 months to IM. Second-generation TKIs initiated were DAS (n = 15), NIL (n = 3), or both sequentially due to intolerance (n = 1). Read More

    Histone deacetylase inhibitors downregulate CCR4 expression and decrease mogamulizumab efficacy in CCR4-positive mature T-cell lymphomas.
    Haematologica 2017 Oct 12. Epub 2017 Oct 12.
    Akita University Graduate School of Medicine, Akita, Japan;
    HDAC inhibitors are promising agents for various T-cell lymphomas, including cutaneous T-cell lymphoma, peripheral T-cell lymphoma, and adult T-cell lymphoma/leukemia. CCR4 is an important therapeutic target molecule because mogamulizumab, an anti-CCR4 antibody, has shown promising efficacy against various T-cell lymphomas. In this study, we examined the in vitro synergistic effects of mogamulizumab and HDAC inhibitors against various T-cell lymphomas. Read More

    Cancer Risk in 2621 Chinese Patients with Inflammatory Bowel Disease: A Population-based Cohort Study.
    Inflamm Bowel Dis 2017 Nov;23(11):2061-2068
    1Department of Medicine and Therapeutics, Prince of Wales Hospital, Hong Kong, China; 2Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China; 3Institute of Digestive Disease, Department of Medicine and Therapeutics, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China; 4Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong, China; 5Department of Medicine, Tuen Mun Hospital, Hong Kong, China; 6Department of Medicine, United Christian Hospital, Hong Kong, China 7Department of Medicine, Princess Margaret Hospital, Hong Kong, China; 8Department of Medicine, Queen Elizabeth Hospital, Hong Kong, China; 9Department of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong, China; 10Department of Medicine, Tseung Kwan O Hospital, Hong Kong, China; 11Department of Medicine, Caritas Medical Centre, Hong Kong, China; 12Department of Medicine, North District Hospital, Hong Kong, China; 13Department of Medicine, Kwong Wah Hospital, Hong Kong, China; 14Department of Medicine, Alice Ho Miu Ling Nethersole Hospital, Hong Kong, China; 15Department of Medicine, Yan Chai Hospital, Hong Kong, China; and 16Department of Gastroenterology, Chelsea and Westminster Hospital, London, United Kingdom.
    Background: Studies on cancer risk in inflammatory bowel disease (IBD) have yielded inconsistent results. We conducted a population-based study to determine the risk of cancer in patients with Crohn's disease (CD) and ulcerative colitis (UC).

    Methods: Using a territory-wide IBD registry in Hong Kong, we identified 2621 patients with IBD and no history of cancer from 1990 to 2016. Read More

    An interesting case of pyoderma gangrenosum with immature hystiocytoid neutrophils.
    J Cutan Pathol 2017 Oct 5. Epub 2017 Oct 5.
    Department of Pathology, Notre-Dame Hospital, University of Montreal, Montreal, Quebec, Canada.
    We present a unique case of a 36-year-old male who developed more than 20 pyoderma gangrenosum (PG) ulcers showing on histopathology a dense inflammatory infiltrate composed of histiocytoid mononuclear immature cells with a strong positivity for myeloperoxidase and Leder stain, suggesting a myeloid lineage in the absence of a concomitant myeloproliferative disorder. Histiocytoid Sweet syndrome (SS) is now recognized as a histological subtype of SS. Although PG and SS belong to the spectrum of neutrophilic diseases, to the best of our knowledge, this is the first case of a "Histiocytoid pyoderma gangrenosum" encompassing immature granulocytes in the absence of leukemia cutis. Read More

    Presentation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma in a Warthin Tumor: Case Report and Literature Review.
    Int J Surg Pathol 2017 Oct 1:1066896917734371. Epub 2017 Oct 1.
    1 Cork University Hospital, Cork, Ireland.
    Warthin tumor is the second most common salivary gland neoplasm. It occurs more commonly in males than in females. Malignant transformation in Warthin tumor is a rare but well-recognized phenomenon; however, the development or presentation of lymphoma in a Warthin tumor is rare. Read More

    Epstein-Barr Virus-Associated Vesiculopapular Eruption on the Face of a Patient with Natural Killer T Cell Lymphoma.
    Ann Dermatol 2017 Oct 25;29(5):618-620. Epub 2017 Aug 25.
    Department of Dermatology, Seoul National University Hospital, Seoul, Korea.
    Unlike typical hydroa vacciniforme (HV), Epstein-Barr virus (EBV)-associated HV-like eruption is more variable in its clinical manifestations. In some patients, progression to lymphoma or leukemia has been reported, which are characterized by the T-cell immunophenotype. Here, we report the first Korean case of EBV-associated vesiculopapular eruption on the face of a patient with natural killer (NK)/T cell lymphoma. Read More

    Higher Expression of Activation-induced Cytidine Deaminase Is Significantly Associated with Merkel Cell Polyomavirus-negative Merkel Cell Carcinomas.
    Yonago Acta Med 2017 Sep 15;60(3):145-153. Epub 2017 Sep 15.
    §Division of Molecular Pathology, Department of Pathology, Tottori University Faculty of Medicine, Yonago 683-8503, Japan.
    Background: Merkel cell carcinomas (MCCs), clinically aggressive neuroendocrine skin cancers, are divided into Merkel cell polyomavirus (MCPyV)-positive and -negative tumors, which show different clinicopathological features and may develop through different mechanisms of carcinogenesis. Aberrant expression of activation-induced cytidine deaminase (AID) as a genomic modulator was demonstrated through pathogen-related NF-κB signal in Helicobacter pylori-associated gastric cancer, adult T cell leukemia/lymphoma (HTLV-1), hepatoma (HCV), and Burkitt lymphoma (EBV).

    Methods: To elucidate the relation of aberrant AID expression in MCPyV-positive and -negative MCCs, we evaluated immunohistochemical expressions of AID and AID-regulating factors between 24 MCPyV-positive and 17 MCPyV-negative MCCs. Read More

    [Clinical characteristics of 6 patients with blastic plasmacytoid dendritic cell neoplasm].
    Zhonghua Xue Ye Xue Za Zhi 2017 Aug;38(8):700-705
    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing 100730, China.
    Objective: To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm. Method: Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed. Results: Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) . Read More

    Case for diagnosis. Infective dermatitis associated with HTLV-1: differential diagnosis of atopic dermatitis.
    An Bras Dermatol 2017 Jul-Aug;92(4):573-574
    Dermatology Service, Hospital das Clínicas, Universidade Federal de Minas Gerais - Belo Horizonte(MG), Brazil.
    Infective dermatitis associated with HTLV-1 (IDH) is the main cutaneous marker of HTLV-1 infection. This disease occurs primarily in children and should be differentiated from other eczemas, especially from atopic dermatitis. The largest series of IDH are from Jamaica and Brazil. Read More

    Midostaurin: a magic bullet that blocks mast cell expansion and activation.
    Ann Oncol 2017 Oct;28(10):2367-2376
    Stanford University School of Medicine/Stanford Cancer Institute, Stanford, USA.
    Clinically relevant features in patients with systemic mastocytosis (SM) include the cosmetic burden of lesional skin, mediator-related symptoms, and organ damage resulting from mast cell (MC) infiltration in advanced forms of SM. Regardless of the SM variant, expansion of neoplastic MC in the skin and other organs is triggered by mutant forms of KIT, the most prevalent being D816V. Activation of MC with subsequent release of chemical mediators is often caused by IgE-dependent mechanisms in these patients. Read More

    Chronic Myeloid Leukemia-the Promise of Tyrosine Kinase Inhibitor Discontinuation.
    Curr Hematol Malig Rep 2017 Oct;12(5):415-423
    Division of Hematology and Oncology, Department of Medicine, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI, 53226, USA.
    Some believe that tyrosine kinase inhibitor (TKI) therapy is as close to perfect as it gets in oncologic therapy. Patients diagnosed with chronic myeloid leukemia (CML) are treated with a daily oral therapy, through which most achieve remission. TKI therapy is not associated with classic chemotherapy side effects, and most patients are able to resume their normal activities of daily living. Read More

    Cutaneous Richter Syndrome Mimicking Primary Cutaneous CD4-Positive Small/Medium T-cell Lymphoma: Case Report and Review of the Literature.
    Am J Dermatopathol 2017 Sep 12. Epub 2017 Sep 12.
    Serviço de Anatomia Patológica, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal.
    Richter syndrome represents the transformation of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma into an aggressive large B-cell lymphoma. Skin involvement is exceptionally rare, with <20 cases reported and its presence as the first presentation of CLL/small lymphocytic lymphoma, as an isolated skin lesion has never been described. Primary cutaneous CD4-positive small/medium T-cell lymphoma (CD4 PCSM-TCL) characteristically presents with a dense dermal infiltrate consisting primarily of small-/medium-sized pleomorphic T-cells and less than 30% large pleomorphic cells. Read More

    Double deficiency of Trex2 and DNase1L2 nucleases leads to accumulation of DNA in lingual cornifying keratinocytes without activating inflammatory responses.
    Sci Rep 2017 Sep 19;7(1):11902. Epub 2017 Sep 19.
    Departament de Patologia i Terapèutica Experimental, Facultat de Medicina i Ciències de la Salut, IDIBELL, Universitat de Barcelona, L'Hospitalet de Llobregat, Barcelona, Spain.
    The cornification of keratinocytes on the surface of skin and oral epithelia is associated with the degradation of nuclear DNA. The endonuclease DNase1L2 and the exonuclease Trex2 are expressed specifically in cornifying keratinocytes. Deletion of DNase1L2 causes retention of nuclear DNA in the tongue epithelium but not in the skin. Read More

    Contrasting effects of an Mdm2 functional polymorphism on tumor phenotypes.
    Oncogene 2017 Sep 18. Epub 2017 Sep 18.
    Department of Genetics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    MDM2, an E3 ubiquitin ligase, is a potent inhibitor of the p53 tumor suppressor and is elevated in many human cancers that retain wild-type p53. MDM2 SNP309G is a functional polymorphism that results in elevated levels of MDM2 (due to enhanced SP1 binding to the MDM2 promoter) thus decreasing p53 activity. Mdm2(SNP309G/G) mice are more prone to spontaneous tumor formation than Mdm2(SNP309T/T) mice, providing direct evidence for the impact of this SNP in tumor development. Read More

    Malignant transformation of oral leukoplakia in a patient with dyskeratosis congenita.
    Oral Surg Oral Med Oral Pathol Oral Radiol 2017 Oct 12;124(4):e239-e242. Epub 2017 Aug 12.
    Department of Dermatology, Walter Reed National Military Medical Center, Bethesda, MD, USA.
    Dyskeratosis congenita (DC) is a rare, inherited, bone marrow failure syndrome caused by premature telomere shortening. The classic mucocutaneous triad of clinical features comprises reticulated skin pigmentation, nail dysplasia, and oral leukoplakia. Multiple somatic features, including bone marrow failure, pulmonary fibrosis, and liver disease, are also common. Read More

    CUTANEOUS T-CELL LYMPHOMA WITH LYMPH NODE METASTASIS IN AN ADULT ADDAX (ADDAX NASOMACULATUS).
    J Zoo Wildl Med 2017 Sep;48(3):933-936
    A 13-yr-old male addax (Addax nasomaculatus) presented with locally extensive alopecia, slight erythema, and skin thickening on the medial aspect of the left rear leg between the stifle and tarsus. Cutaneous T-cell lymphoma was diagnosed after histopathology and immunohistochemical staining of representative skin-punch biopsies. No treatment was elected, and the addax was euthanized 3 yr later because of poor body condition, chronic dental disease, and confirmed spread of lymphoma to other cutaneous locations. Read More

    Lenalidomide maintenance after first-line therapy for high-risk chronic lymphocytic leukaemia (CLLM1): final results from a randomised, double-blind, phase 3 study.
    Lancet Haematol 2017 Oct 12;4(10):e475-e486. Epub 2017 Sep 12.
    Department I of Internal Medicine and Center of Integrated Oncology Cologne-Bonn, German CLL Study Group, University Hospital of Cologne, Cologne, Germany.
    Background: The combined use of genetic markers and detectable minimal residual disease identifies patients with chronic lymphocytic leukaemia with poor outcome after first-line chemoimmunotherapy. We aimed to assess lenalidomide maintenance therapy in these high-risk patients.

    Methods: In this randomised, double-blind, phase 3 study (CLLM1; CLL Maintenance 1 of the German CLL Study Group), patients older than 18 years and diagnosed with immunophenotypically confirmed chronic lymphocytic leukaemia with active disease, who responded to chemoimmunotherapy 2-5 months after completion of first-line therapy and who were assessed as having a high risk for an early progression with at least a partial response after four or more cycles of first-line chemoimmunotherapy, were eligible if they had high minimal residual disease levels or intermediate levels combined with an unmutated IGHV gene status or TP53 alterations. Read More

    Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review.
    J Pediatr Hematol Oncol 2017 Oct;39(7):528-537
    *Faculty of Medicine §Faculty of Health Sciences, University of Ottawa Departments of †Pediatric Surgery ∥Pediatric Pathology ‡Children's Hospital of Eastern Ontario, Research Institute ¶Department of Pediatrics, Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
    Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. Read More

    A Review of 7 Cases of Laparoscopic Cholecystectomy for Pediatric Cholecystolithiasis.
    J UOEH 2017 ;39(3):223-227
    Department of Surgery 1, School of Medicine, University of Occupational and Environmental Health, Japan.
    Pediatric cholecystolithiasis is a relatively rare disease, but it is recently increasing in Japan. Laparoscopic cholecystectomy (LC) is a standard procedure for cholecystolithiasis not only in adults but also in children, and we are aggressively introducing single-incision laparoscopic cholecystectomy (SILC) at our hospital. We reviewed the patient characteristics, operation procedures and outcomes of 7 children (15 years old and under) with cholecystolithiasis who underwent LC in our hospital between August 1995 and December 2015. Read More

    Myeloid Sarcoma Presenting as Nasal and Orbital Mass: An Initial Manifestation of an Acute Myeloid Leukaemia.
    J Clin Diagn Res 2017 Jul 1;11(7):ED24-ED26. Epub 2017 Jul 1.
    Professor, Department of ENT, Maulana Azad Medical College, New Delhi, India.
    Myeloid sarcoma is an extramedullary manifestation of Acute Myeloid Leukaemia and sometimes is the only indicator of the disease. The incidence varies between 3-9.1% of acute leukaemia cases. Read More

    Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia.
    J Cutan Pathol 2017 Sep 8. Epub 2017 Sep 8.
    Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. Read More

    Successful 5-azacytidine treatment of myeloid sarcoma and leukemia cutis associated with myelodysplastic syndrome: A case report and literature review.
    Medicine (Baltimore) 2017 Sep;96(36):e7975
    aDepartment of Hematology bDepartment of Transfusion Medicine and Regenerative Medicine, Niigata University Medical and Dental Hospital cDepartment of Pathophysiology, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Niigata, Japan.
    Rationale: Myeloid sarcoma (MS) and leukemia cutis (LC) are extramedullary tumors comprising myeloid blasts. They can occur de novo or concurrently with hematological disorders, usually acute myeloid leukemia (AML). AML chemotherapy is generally the initial therapy for MS and LC, and hematopoietic stem cell transplantation (HSCT) can be considered as additional therapy. Read More

    Severe Mucha-Habermann-Like Ulceronecrotic Skin Disease in T-Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant.
    Pediatr Dermatol 2017 Sep;34(5):e265-e270
    Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
    A 5-year-old girl with T-cell acute lymphoblastic leukemia (T-ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha-Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. Read More

    Livedoid eruption in a patient affected by T-γδ large granular lymphocyte leukaemia.
    BMJ Case Rep 2017 Sep 7;2017. Epub 2017 Sep 7.
    Unit of Dermatology, University of Padua, Padova, Italy.
    Livedo is an ischaemic dermopathy characterised by a reddish-blue to violaceous mottling of the skin with a net-like reticular appearance. Livedo has been described in association with several medical conditions including lymphoproliferative disorders. Here, we describe the case of a 60-year-old woman who was presented with asymptomatic and persistent livedoid eruption on her trunk, lower and upper extremities as manifestation of an indolent form of T-γδ large granular lymphocyte leukaemia. Read More

    Indeterminate dendritic cell neoplasm of the skin: A 2-case report and review of the literature.
    J Cutan Pathol 2017 Nov 6;44(11):958-963. Epub 2017 Sep 6.
    Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas.
    Indeterminate dendritic cell neoplasm (IDCN) is an exceedingly rare and mostly cutaneous histiocytosis, frequently associated with other hematopoietic malignancies. We report 2 cases of multilesional cutaneous IDCN. A 55-year-old male with no associated malignancy and complete response to ultraviolet phototherapy; and a 72-year-old male with chronic myelomonocytic leukemia (CMML). Read More

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