Intravascular lymphoma (IVL) is a rare extranodal non-Hodgkin lymphoma. We performed a retrospective analysis of 55 IVL patients who were treated at our institution 2003-2018. Median age at diagnosis was 68 years, and 64% were males.
View Article and Find Full Text PDFPurpose: This study compares reduced (<27 Gy) to standard dose (≥30 Gy) radiation therapy (RT) in the treatment of gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (gMALT lymphoma).
Methods And Materials: Forty-two patients with stage I or II disease were retrospectively reviewed. Response to RT was assessed with endoscopy after RT.
Dermatopathology (Basel)
June 2021
Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an extremely rare and aggressive T-cell neoplasm with complex heterogeneity. We present a series of two patients who presented with firm, subcutaneous nodules and were diagnosed with PCGD-TCL. In both cases, biopsies demonstrated a both superficial and deep adnexotropic infiltrate comprised of angiocentric, medium- to large-sized atypical lymphocytes.
View Article and Find Full Text PDFEBV-positive HHV8-negative EBL is part of the spectrum of EBV-positive diffuse large B-cell lymphoma NOS. This entity can be labeled as primary age-related EBV-associated EBL and appears to respond well to rituximab and thoracentesis.
View Article and Find Full Text PDFMyeloid sarcoma, also known as chloroma or granulocytic sarcoma is an extramedullary disease process that typically presents in association with acute myeloid leukemia during initial presentation or at relapse. Often associated with cytogenetic mutations, including t(8;21)(q22;q22); , and less frequently with inv(16)(p13.1q22) or t(16;16)(p13.
View Article and Find Full Text PDFInterleukin 6 receptor (IL6R) inhibitor, tocilizumab, has been effectively used in the treatment of cytokine release syndrome in patients receiving chimeric antigen receptor T-cell therapy. Here we present a patient with chronic myelomonocytic leukemia (CMML) who developed a steroid refractory, post-operative myelomonocytic leukemoid reaction (PO-MMLR), effectively treated with tocilizumab. Although, further studies are needed to validate the effectiveness of tocilizumab in management of PO-MMLR, this case serves to provide a new management approach in treatment of this rare but lethal syndrome with no standardized treatment options.
View Article and Find Full Text PDFClin Lymphoma Myeloma Leuk
February 2021
Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis.
Patients And Methods: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes.
This case illustrates an unusual presentation of paraneoplastic myositis in stage IV follicular lymphoma managed with high-dose steroids, a previously unreported entity in the literature.
View Article and Find Full Text PDFConcurrent presentation of acute promyelocytic leukemia (APL) with other hematologic diseases in the absence of previous chemotherapy or ionizing radiotherapy treatment is very rare. We present a case of simultaneous occurrence of APL with myelodysplastic syndrome (MDS)-related acute myeloid leukemia (AML). A 43-yearold female presented with 3 month of history fatigue, night sweats, chills and pancytopenia.
View Article and Find Full Text PDFGiven the prevalence of breast cancer and the mortality associated with metastatic disease, it is imperative for physicians to not only be aware of common sites but also of rare metastatic destinations such as the bladder. A postmenopausal woman with a medical history of stage 2 invasive ductal carcinoma, oestrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 negative, in remission for 9 years, presented to her primary care physician with concerns of increased urinary urgency, frequency and incontinence. The patient underwent cystoscopy with biopsy of an area of granulation tissue.
View Article and Find Full Text PDFWe previously reported an extremely rare case of follicular dendritic cell sarcoma (FDCS) presented as a thyroid mass. Given the rarity of this disease, there are no personalized and molecularly targeted treatment options due to the lack of knowledge in the genomic makeup of the tumor. A 44-year-old white woman was diagnosed with an extranodal FDCS in thyroid.
View Article and Find Full Text PDFHairy cell leukemia (HCL) is a low grade B-cell lymphoproliferative disorder that typically presents with splenomegaly, cytopenias, and diffuse bone marrow infiltration. There have been few cases in the literature of HCL presenting as lymphomas in extra-nodal locations, such as soft tissues and bones without circulating leukemic cells, splenomegaly, or iliac crest bone marrow involvement. We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases.
View Article and Find Full Text PDFMantle cell lymphoma (MCL) is typically characterized by t(11;14), which places the IGH@ enhancer elements upstream of CCND1. This fusion results in up-regulation of CCND1 and consequently its protein product cyclin D1. Recent studies have shown that in MCL, mutations or translocations occurring within the 3' untranslated region (UTR) of the CCND1 gene can result in a truncated mRNA transcript that is more stable and associated with more aggressive disease.
View Article and Find Full Text PDFHuman herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders.
View Article and Find Full Text PDFGiant cell myocarditis (GCM) is a rare and commonly fatal form of fulminant myocarditis. During the acute phase, while immunosuppressive therapy is initiated, venoarterial extracorporeal membrane oxygenation (VA-ECMO) support is commonly used as a bridge to heart transplantation or recovery. Until recently, conventional transesophageal echocardiography and transthoracic echocardiography were the tools available for hemodynamic assessment of patients on this form of mechanical circulatory support.
View Article and Find Full Text PDFPrimary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a rare diffuse large B-cell lymphoma confined to the skin of the legs. The typical presentation is characterized by solitary or multiple growing plaques, usually confined to one leg. We report a case of PCDLBCL-LT of activated B-cell subtype characterized by multiple local relapses in the legs, initially, and systemic relapses about seven years after the diagnosis.
View Article and Find Full Text PDFOtolaryngologists are called upon to evaluate and treat sinonasal masses discovered incidentally on imaging studies. Although common conditions such as sinonasal polyps and mucus retention cysts predominate, it is prudent practice to formulate a differential diagnosis to identify unusual conditions. We present a case of a maxillary sinus mass in a 78-year-old man that was discovered incidentally on brain imaging and subsequently identified on biopsy as an angiomyolipoma (AML).
View Article and Find Full Text PDFGastrointestinal tumors can rarely cause intestinal intussusception. Herein, we describe a 74 year-old male with a presumed diagnosis of Crohn's disease who presented with persistent symptoms refractory to medical management. Radiography demonstrated small bowel intussusception into the cecum.
View Article and Find Full Text PDFA 69-year-old man was admitted to our hospital with left upper quadrant pain, splenomegaly, acute renal failure, hyperuricemia, thrombocytopenia (platelet count 32,000/mm), and leukocytosis (leukocyte count 88,000/mm). His medical history was significant for prior diagnosis of myelodysplastic syndrome (MDS) by bone marrow biopsy at an outside institution treated with the hypomethylating chemotherapeutic agent decitabine, atrial fibrillation, cardiomyopathy, and complete pacemaker dependence. Neurologic examination upon admission was normal.
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