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Myeloid dermatosis with features of sweet syndrome and leukemia cutis: a case report.

Occult hematologic malignancies can initially present with cutaneous manifestations. We describe a 53-year-old woman with chronic hepatitis C and intravenous drug use presenting with a painful, progressive leg rash and worsening bicytopenias. A skin punch biopsy revealed neutrophilic dermatosis morphologically compatible with Sweet syndrome (SS), along with rare CD117+/CD34 − blastoid cells. Subsequent bone marrow studies revealed acute myeloid leukemia with mutated NPM1. This case highlights the diagnostic challenge of distinguishing SS from leukemia cutis (LC) when skin biopsies demonstrate

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm with Orbital Tumor as the Initial Symptom.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy arising from precursor dendritic cells. It is a rare and challenging clinical presentation. For decades, there has been no treatment course for managing BPDCN and its overall prognosis is poor. METHODS AND RESULTS: We report a 27-year-old man who was admitted to the hospital due to an orbital tumor as the first symptom. Progressive enlargement of the orbital tumor was accompanied by multiple purple circular nodules on the body trunk. Pathological confirmation of BPDCN after resection of the orbit

Autoimmune neutropenia preceding Helicobacter pylori-negative MALT lymphoma with nodal dissemination.

Autoimmune neutropenia (AIN), resulting from granulocyte-specific autoantibodies, is much less frequent than other autoimmune hematologic disorders including autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). These autoimmune disorders may precede, synchronize, or follow collagen disorders, viral infections, and lymphoid neoplasms. Herein we present the first case of AIN in association with Helicobacter pylori-negative mucosa-associated lymphoid tissue (MALT) lymphoma with nodal dissemination. In our case, AIN, accompanied by ITP, occurred prior to the clinical manifestation

An adult patient with systemic mastocytosis and B-acute lymphoblastic leukemia.

Mastocytosis is a myeloproliferative neoplasm characterized by clonal expansion of abnormal mast cells, ranging from the cutaneous forms of the disease to mast cell leukemia. In a significant proportion of patients, systemic mastocytosis (SM) coexists with another hematologic malignancy, termed systemic mastocytosis with an associated hematologic nonmast cell lineage disorder (SM-AHNMD). Despite the pronounced predominance of concomitant myeloid neoplasms, the much more unusual coexistence of lymphoproliferative diseases has also been reported. Imatinib mesylate (IM) has a role in the treatmen

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… A form of reactive mast cell hyperplasia, which is observed in other hematologic neoplasms, such as lymphoplasmacytic lymphoma and hairy cell leukemia (HCL) must be excluded [ 3 ]. …

[Proteins in haematology].

The introduction of monoclonal antibodies has been a milestone in the treatment of hematologic neoplasms. The CD20 antibody rituximab has been a trailblazer and represents meanwhile a fixed combination partner of different first- and second-line chemotherapies in CD20-positive B-cell-non-Hodgkin's lymphoma (NHL). Rituximab maintenance is established in follicular lymphoma. The drug is also used for the treatment of non-malignant hematologic diseases (i.e. immunthrombocytopenia). Rituximab was followed by the CD52 antibody alemtuzumab and recently by the CD20 antibody ofatumumab for the therapy…

Red blood cell alloimmunization in transfused oncology patients at a high-complexity center in Colombia.

BACKGROUND: Red blood cell transfusion is essential for managing anemia in cancer patients but can induce erythrocyte alloimmunization, particularly against Rh (anti-E) and Kell (anti-K) antigens, which complicates transfusion compatibility and increases the risk of hemolytic events. This study assessed the prevalence and profile of irregular antibodies in transfused oncology patients at a high-complexity center. METHODS: A retrospective descriptive study analyzed medical records of 179 adult patients with solid tumors or hematologic neoplasms who received red blood cell transfusions. Demogra…

Standardized bone marrow assessment, risk variables, and survival in dogs with myelodysplastic syndrome and acute myeloid leukemia.

Myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) are heterogeneous neoplasms of hematopoietic stem cells that are challenging to diagnose, differentiate, and prognosticate. Cytogenetic and mutational analyses are useful in humans but unavailable for dogs, where diagnosis and classification still rely largely on hematologic and morphologic assessment. The objectives of this study were to apply a classification scheme to myeloid neoplasms and to assess outcome in relation to predictor variables. Keyword search of a laboratory database, application of sequential exclusion criteria,

Preliminary Cytomorphologic Diagnosis of Hematolymphoid Malignancies in Effusions: A Cyto-histo Correlation with Lessons on Restraint.

BACKGROUND: Effusions as part of hematologic neoplasms are rare and as a primary presentation, rarer. In standalone laboratories of developing countries, resorting to techniques such as flow cytometry or immunohisto/cytochemistry may not be possible. A near definitive diagnosis on cytomorphology would, therefore, be an ideal beginning. To that end, we compiled our cases of primary hematolymphoid effusions, devising reproducible reporting categories and looked at their concordance with the final histopathology. SUBJECTS AND METHODS: Fifty-four cases of primary hematolymphoid effusions over 10 …

fuPCR as diagnostic method for the detection of rare fungal pathogens, such as Trichosporon, Cryptococcus and Fusarium.

Fungal respiratory tract colonization is a common finding in patients with hematologic neoplasms due to immunosuppression inherent in the diseases and exacerbated by therapy. This greatly increases the risk of fungal infections of the lungs, which is associated with significant mortality. Therefore, reliable diagnostic methods with rapidly available results are needed to administer adequate antifungal therapy. We have established an improved method for fungal DNA extraction and amplification that allows simultaneous detection of fungal families based on a set of multiplexed real-time PCR react…

Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms.

Philadelphia-negative classical myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The 2016 revision of the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues includes new criteria for the diagnosis of these disorders. Somatic mutations in the 3 driver genes, that is, JAK2, CALR, and MPL, represent major diagnostic criteria in combination with hematologic and morphological abnormalities. PV is characterized by erythrocytosis with suppressed endogenous erythropoietin production, bone marrow panmy

Blastic Plasmacytoid Dendritic Cell Neoplasm Developed in Chronic Myeloid Leukemia in Molecular Remission During a Four-Year Treatment-Free Interval After Six Years of Dasatinib Treatment.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy affecting multiple sites, most commonly the skin. About 10-20% of BPDCN cases are accompanied by hematological neoplasms. A 71-year-old male was diagnosed with chronic myeloid leukemia in the chronic phase (CML-CP) 11 years prior (at 60 years of age), and dasatinib treatment was initiated. A major molecular response (MMR) was achieved 18 months after diagnosis, and the molecular response (MR)4.0 lasted beyond 36 months. Due to pancytopenia, dasatinib was discontinued at 74 months, but the CML-CP remained un

Systemic Mastocytosis: Advances in Diagnosis and Current Management.

Mastocytosis is a rare hematologic disorder characterized by abnormal proliferation and accumulation of neoplastic mast cells in various body sites. Isolated skin involvement is termed cutaneous mastocytosis (CM) and the term systemic mastocytosis (SM) refers to multi-organ involvement, most commonly of the bone marrow, skin, liver, and spleen. A subset of patients with SM have an associated clonal hematologic neoplasm which is most commonly myelodysplastic syndrome, chronic myelomonocytic leukemia, or acute myelogenous leukemia and this entity is termed SM with associated hematological neopla

Increased Prevalence of Extrathymic Neoplasms in Myasthenia Gravis Patients-A Population-Based, Matched Case-Control Study.

INTRODUCTION/AIMS: Myasthenia gravis (MG) is associated with thymic neoplasms. However, an increased prevalence of extrathymic neoplasms has … ncrease the risk of malignancy. DISCUSSION: MG patients have a higher prevalence of both solid and hematologic neoplasms compared to non-myasthenic controls, regardless of immunosuppressive treatment. This supports the notion that malignancy is related to MG disease itself rather than external factors.

Prevalence of Merkel cell polyomavirus DNA in cutaneous lymphomas, pseudolymphomas, and inflammatory skin diseases.

Several groups confirmed Merkel cell polyomavirus (MCPyV) as the likely causative agent of Merkel cell carcinoma. Hematolymphoid disorders are known to be a substantial risk factor for Merkel cell carcinoma, and vice versa. The association between MCPyV and hematologic neoplasms is poorly analyzed, as well as the speculation that lymphocytes may serve as reservoir for MCPyV. Therefore, we investigated the prevalence of MCPyV DNA in primary cutaneous T- and B-cell lymphomas, pseudolymphomas (PLs), and inflammatory skin diseases with dominant lymphocytic infiltrate. We performed a molecular path…

Risk of Head and Neck Cancer in Patients With Prior Hematologic Malignant Tumors.

IMPORTANCE: More than 1.3 million people in the United States have a hematologic malignant tumor currently or in remission. Previous studies have demonstrated an increased risk of secondary neoplasms in patients with hematologic malignant tumors, but research specifically on the risk of head and neck solid tumors in patients with prior hematologic malignant tumors is limited. OBJECTIVES: To examine a possible association between prior hematologic malignant tumors and risk of head and neck cancer and to assess the overall survival (OS) among these patients. DESIGN, SETTING, AND PARTICIPANTS:

Symptoms of Hematologic Tumors Patients after CAR-T Therapy: A Systematic Review and Meta-Analysis.

CONTEXT: Patients with hematologic neoplasms after Chimeric antigen receptor T-cell (CAR-T) therapy have multiple syndromes, with corresponding symptoms. OBJECTIVES: The review aimed to integrate the severity and incidences of symptoms in these patients, and to investigate the difference of the symptoms among different geographic locations, types of hematological tumors, evaluating instruments, and evaluating time, to provide a theoretical basis for symptom management. METHODS: A literature search of PubMed, Web of Science, Embase, the Cochrane Library, China National Knowledge Internet, Sin…

Russell Body Lesions of the Colon: Case Report and Literature Review.

Russell bodies (RB) are rare manifestations within the lower gastrointestinal tract. To date, there are only three other reported cases of RB lesions of the colon; two were polyps, and the third was a case of a multifocal RB lesion of the gastrointestinal tract. This paper reports a case of a tubulovillous adenoma with RB of the sigmoid colon in a patient diagnosed incidentally as part of the UK National Health Service Bowel Cancer Screening Programme. A thorough hematological investigation is required to exclude hematological malignancies because of its association with plasma cell neoplasm.

The Current Landscape of Pulmonary Hypertension in Myeloproliferative Neoplasms.

Pulmonary hypertension (PH) is an increasingly recognized complication of Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). Recent data suggest prognostic implications of both cardiovascular morbidity and mortality and hematologic progression. Additionally, PH and MPNs share common pathophysiologic mechanisms that bridge these two seemingly different disorders. The landscape of PH in MPNs is evolving in the face of improved survivorship and reduced disease progression, leading to increased incidence and prevalence of concurrent and/or consequent cardiovascular disease. Pros

From Spores to Solutions: A Comprehensive Narrative Review on Mucormycosis.

Mucormycosis is an infrequent but fatal illness that mainly affects patients with uncontrolled diabetes mellitus, diabetic ketoacidosis, solid and hematologic neoplasms, organ transplantation, chronic steroid intake, prolonged neutropenia, iron overload states, neonatal prematurity, severe malnutrition, and HIV. Many cases were reported across the world recently following the COVID-19 pandemic. Recent research has led to a better understanding of the pathogenesis of the disease, and global guidelines are now available for managing this serious infection. Herein, we comprehensively review the e…

Composite Histiocytic Sarcoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma of the Ocular Adnexa.

Histiocytic sarcoma (HS) is a rare and aggressive hematologic neoplasm characterized by the proliferation of malignant histiocytes. It infrequently presents with periorbital involvement. Here we present the first documented case of ocular adnexal histiocytic sarcoma composite with chronic lymphocytic leukemia/small lymphocytic lymphoma and provide compelling evidence for the transdifferentiation of chronic lymphocytic leukemia/small lymphocytic lymphoma to histiocytic sarcoma in an 80-year-old woman. Comprehending the clinicopathological characteristics of histiocytic sarcoma and various other