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
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
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
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
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
*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
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
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 (CMML) is a clonal hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features and characterized by persistent relative and absolute monocytosis in the peripheral blood. Read More
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
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
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 (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 light therapy; and a 72-year-old male with chronic myelomonocytic leukemia (CMML). Read More
Background: Cutaneous involvement is more prevalent in B lymphoblastic lymphoma (B-LBL) than T lymphoblastic lymphoma (T-LBL). The authors describe a rare case of cutaneous involvement in a 25-year-old woman with T lymphoblastic lymphoma leukemia.
Methods: Hematologic investigation, bone marrow aspirate and biopsy, cytogenetic analysis and cutaneous lesion biopsy were performed. Read More
Département de Génétique, AP-HP Hôpital Pitié-Salpêtrière, UPMC Univ Paris 06, Paris, France.
This review focuses on the classification, diagnosis and natural history of congenital neutropenia (CN). CN encompasses a number of genetic disorders with chronic neutropenia and, for some, affecting other organ systems, such as the pancreas, central nervous system, heart, bone and skin. To date, 24 distinct genes have been associated with CN. Read More
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Read More
Pyoderma gangrenosum is an intractable disease of unknown cause involving recurrent ulcerative lesions on the skin, and may accompany ulcerative colitis, rheumatoid arthritis, leukemia, systemic lupus erythematosus, and other conditions. Here, we report a rare case of pyoderma gangrenosum in the thoracic abdomen following post-mastectomy reconstructive surgery. A 39-year-old presented at the hospital with a complaint of left papilla erosion. Read More
Brain Bank for Aging Research, Department of Neuropathology, Department of Neurology, and Department of Pathology; Tokyo Metropolitan Geriatric Hospital and Institution of Gerontology, Tokyo, Japan; Department of Neurology and Clinical Neuroscience; and Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
Neuronal intranuclear hyaline inclusion disease (NIHID) is a neurodegenerative disorder characterized by the presence of eosinophilic nuclear inclusions (NIs) in diverse cell lines in systemic organs. Adult-onset NIHID typically manifests with dementia associated with leukoencephalopathy. The detection of NIs in skin biopsies is useful for an antemortem diagnosis. Read More
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly characterized, rare malignant tumor of the skin and hematopoietic system. BPDCN occurs mainly in the elderly, whereas it is rarer among children, and has variable clinical manifestations. Optimal chemotherapeutic regimens for the treatment of BPDCN have not yet been determined and this tumor has a poor prognosis. Read More
Tyrosine kinase enzymes are an attractive target for anticancer therapies. Tyrosine kinase inhibitors (TKI) are well tolerated; somehow severe systemic side effects are rarely seen during treatment. Toxicities of skin and appendages may lead to poor compliance, psychosocial inconvenience, and drug interruption. Read More
Background: The roots of Eurycoma longifolia Jack have traditionally been used as an aphrodisiac tonic besides the other remedies for boils, fever, bleeding gums, and wound ulcer. Recently, the antiproliferative activity of E. longifolia has been reported and remained attractive to natural chemists. Read More
Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement. Read More
Background: l-Asparaginase is essential in the treatment of childhood acute lymphoblastic leukemia. If immunoglobulin G anti-l-asparaginase antibodies develop, they can lead to faster plasma clearance and reduced efficiency as well as to hypersensitivity reactions, in which immunoglobulin E can also participate. This study investigated the presence of immunoglobulin G and immunoglobulin E anti-l-asparaginase antibodies and their clinical associations. Read More
The harmful effects of oil on various species of flora and fauna have been studied extensively; however, few studies have studied the effects of oil exposure on human health. The objective of this research was to collect information on the acute health effects and serious psychological symptoms of the possible consequences of such exposure to crude oil. Some studies focused on the composition of different chemicals used in the extraction process, and wastes generated proved to be highly harmful to human health. Read More
Background: To evaluate cancer incidence among licenced commercial pilots in association with cosmic radiation.
Methods: Cohort study where ionizing radiation dose of cosmic radiation was estimated from airline data and software program and cancer incidence was obtained by record linkage with nation-wide cancer registry. All licenced commercial male airline pilots were followed from 1955 to 2015, ever or never employed at airline with international routes. Read More
We describe the case of a patient with Philadelphia-positive acute lymphoblastic leukemia treated with dasatinib plus steroids as first-line therapy, who achieved a major molecular response (MMR) before undergoing matched, unrelated donor allogeneic stem cell transplant. Eleven months after the transplant, she experienced molecular relapse. Mutational screening showed negativity for the T315I mutation, The patient underwent a salvage chemotherapy regimen with clofarabine + cyclophosphamide + steroids and ponatinib (clofarabine 70 mg i. Read More
Introduction: Extended survival outcomes from improved treatments for patients with cancer come with an increased risk of developing a metachronous second malignancy (MSM). We evaluated the incidence of MSM after successful treatment of SCLC and compared survival between SCLC patients who developed MSM and those who did not.
Methods: Selection criteria were a diagnosis of limited-stage SCLC and receipt of ≥45 Gy radiotherapy and chemotherapy at a single institution in 1985-2012. Read More
Department of Dermatology, Yale Dermatopathology Laboratory, 15 York Street, LMP5031, New Haven, CT 06520, USA; Department of Pathology, Yale Dermatopathology Laboratory, 15 York Street, LMP5031, New Haven, CT 06520, USA. Electronic address:
The classification of myeloid neoplasms has undergone major changes and currently relies heavily on genetic abnormalities. Cutaneous manifestations of myeloid neoplasms may be the presenting sign of underlying bone marrow disease. Dermal infiltration by neoplastic cells may occur in otherwise normal skin or in sites of cutaneous inflammation. Read More
Introduction: Bendamustine, typically in combination with rituximab, is an effective treatment for chronic lymphocytic leukemia (CLL) and B-cell non-Hodgkin lymphoma. Despite its acceptable short-term toxicity profile, long-term toxicities are less well established. This study investigated the long-term adverse effects of bendamustine and responses to subsequent treatments. Read More
Bullous Sweet's syndrome is a rare variant of the inflammatory neutrophilic dermatosis characterised by painful bullous skin lesions, fever, leukocytosis and a neutrophilic infiltrate of the dermis. The condition may be classified according to aetiology into classical (idiopathic), malignancy-associated and drug-induced. Neutrophilic infiltration occurs in response to a systemic insult. Read More
A 73-year-old woman presented a 3-year history of indolent enlargement of cutaneous tumor nodules. Peripheral blood flow cytometry revealed thrombocytopenia (platelets; 85,000/µl) and the presence of an abnormal, small B lymphocyte population (CD5(+), CD10(-), CD20(+), CD22(+), CD23(dim), FMC7(+), SmIgλ(+), and SmIgκ(-); 4,000/µl). Skin biopsy indicated infiltration of CD5(+), CD10(-), CD20(+), BCL2(+), BCL6(+), and cyclin D1(-) atypical large B-cells, suggesting diffuse large B-cell lymphoma. Read More
The present study sought to elucidate the prognosis of adult T-cell leukemia-lymphoma (ATL) patients receiving mogamulizumab, a defucosylated anti-CCR4 monoclonal antibody. Progression-free survival (PFS) and overall survival (OS) of ATL patients enrolled in two studies are herein updated, namely NCT00355472 (phase I study of mogamulizumab in relapsed patients with ATL and peripheral T-cell lymphoma) and NCT00920790 (phase II study for relapsed ATL). Of 13 patients with relapsed aggressive ATL in the phase I study, four (31%) survived >3 years. Read More
Mastocytosis refers to a heterogeneous group of disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin (cutaneous mastocytosis when only in the skin, CM) or in various organs (systemic mastocytosis, SM). This leads to a wide variety of clinical manifestations resulting from excessive mediator release in CM and benign forms of SM (indolent SM, ISM) and from tissue mast cell infiltration causing multiorgan dysfunction and failure in more aggressive subtypes (aggressive SM, ASM, or mast cell leukemia). In addition, SM may be associated with hematological neoplasms (AHN). Read More
A 14-year-old boy who had undergone a matched sibling bone marrow transplant for acute lymphoblastic leukemia presented with painful nodules on his palms after prolonged gaming on his computer and mobile phone. Histology showed a neutrophilic inflammatory infiltrate surrounding the acrosyringium and eccrine sweat coils in the deep dermis. The lesions resolved spontaneously with conservative management. Read More
Purpose: The purpose of the paper is to assess the influence of marital status on conditional relative survival of cancer according to sex.
Methods: Analyses involved 779,978 males and 1,032,868 females diagnosed with 1 of 13 cancer types between 2000 and 2008, and followed through 2013. Data are from the Surveillance, Epidemiology, and End Results (SEER) Program. Read More
The roots of Euphorbia kansui, which belong to the family Euphorbiaceae, have been used as a traditional medicine for the treatment of various diseases such as diabetes, ascites, and leukemia. Recently, it was reported that the methylene chloride fraction of E. kansui radix (EKC) regulated the differentiation of Th17 cells and alleviated the symptoms of Th17-related inflammatory bowel disease. Read More
Extramedullary plasmacytoma (EMP) accounts for only 3% of plasma cell malignancies; others include multiple myeloma, plasma cell leukemia and solitary plasmacytoma of bone. The majority of EMPs are found in the upper respiratory tract. Other sites include the GI tract, bladder, CNS, thyroid, breast, testes, parotid gland, lymph nodes and skin. Read More
We present a case of a 61-year-old Caucasian woman who was hospitalized with fevers on day 176 after a matched unrelated stem cell transplant for acute myelogenous leukemia. She developed hemorrhagic bullae on the skin of her right thigh, and blood cultures and skin biopsy both confirmed Fusarium proliferatum. Despite antifungal therapy, her condition worsened and she died while on comfort-only measures. Read More
Cutaneous manifestations of myeloid malignancies are common and have a broad range of presentations. These skin findings are classified as specific, due to direct infiltration by malignant hematopoietic cells, or non-specific. Early recognition and diagnosis can have significant clinical implications, as skin manifestations may be the first indication of underlying hematologic malignancy, can reflect the immune status and stage of disease, and cutaneous reactions may occur from conventional and targeted agents used to treat myeloid disease. Read More
Mast cells (MCs) are physiologically activated by binding of stem cell factor (SCF) to the extracellular domains of the Kit receptor. This binding increases the proliferation and prolongs the survival of normal mature MCs, as well as intensifies the release of mediators. In mastocytosis, somatic mutations of the coding Kit gene cause autocrine dysregulation and lead to constitutive KIT activation even in the absence of its ligand SCF. Read More
aPain Research Center, Department of Anesthesiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA bDepartments of Pharmacology and Cell Biophysics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Vincristine (VNC) is commonly used to treat pediatric cancers, including the most prevalent childhood malignancy, acute lymphoblastic leukemia. Although clinical evidence suggests that VNC causes peripheral neuropathy in children, the degree to which pediatric chemotherapeutic regimens influence pain sensitivity throughout life remains unclear, in part because of the lack of an established animal model of chemotherapy-induced neuropathic pain during early life. Therefore, this study investigated the effects of VNC exposure between postnatal days (P) 11 and 21 on mechanical and thermal pain sensitivity in the developing rat. Read More
Introduction: With the aim of providing cancer control indicators, this work presents cancer survival in adult (≥15 years) patients in Spain diagnosed during the period 2000-2007 from Spanish cancer registries participating in the EUROCARE project.
Methods: Cancer cases from nine Spanish population-based cancer registries were included and analysed as a whole. All primary malignant neoplasms diagnosed in adult patients were eligible for the analysis. Read More
Background: With the improving outcomes of patients with follicular lymphoma (FL), it is imperative to focus on survivorship issues, including the development of second primary malignancies (SPMs). We used a large US database to measure the risk of SPMs among FL survivors.
Materials And Methods: We used the Surveillance, Epidemiology, and End Results-13 registry to identify FL patients from 1992 to 2011. Read More