Polymorphic lymphoproliferative disorder arising in a rheumatoid arthritis patient, presenting as fibrin-associated large B-cell lymphoma-like lesions in aortic and mitral valves.

We herein report a case of methotrexate-associated lymphoproliferative disorder (MTX-LPD) showing fibrin-associated large B-cell lymphoma-like heart valve lesions, and Epstein-Barr virus (EBV)-positive mucocutaneous ulcer-like cutaneous and oral mucosal lesions. MTX-LPD is a critical complication that can occur in RA patients who are treated with MTX. EBV also plays a defining or important role in LPDs.

Insulin-like Growth Factor II mRNA-binding Protein 3 is a Highly Sensitive Marker for Intravascular Large B-cell Lymphoma: Immunohistochemical Analysis of 152 Pathology Specimens From 88 Patients.

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of aggressive extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of blood vessels, particularly capillaries. IVLBCL lacks mass formation, and its diagnosis can be challenging. We analyzed the utility of insulin-like growth factor II mRNA-binding protein 3 (IMP3) immunohistochemistry for IVLBCL diagnosis in various organs.

Pheochromocytoma Multisystem Crisis Complicated by Severe Acute Pancreatitis.

A 43-year-old man developed headache, dizziness, abdominal pain, and vomiting. His blood pressure was 203/121 mmHg, heart rate 122 beats/min, body temperature 39.1°C, and respiratory rate 24/min.

An extremely rare case of anterior mediastinal leiomyosarcoma: a case report.

We herein report an extremely rare case of leiomyosarcoma found in the anterior mediastinum. A 79-year-old man presented to our hospital with an anterior mediastinal mass incidentally found by chest computed tomography (CT) scan. Percutaneous needle biopsy revealed the presence of an undifferentiated sarcoma.

Primary Hepatic Neuroendocrine Carcinoma with Thrombocytopenia Due to Diffuse Bone Marrow and Splenic Infiltration: An Autopsy Case.

An 82-year-old man with fever and back pain was referred to our hospital and was thus found to be thrombocytopenic. A bone marrow biopsy revealed the diffuse infiltration of poorly differentiated neuroendocrine carcinoma (NEC). Computed tomography revealed a large hepatic mass.

Immunohistochemistry of the oncofetal protein IMP3 is helpful in the diagnosis of intravascular large B-cell lymphoma using skin biopsy.

Background: The oncofetal protein insulin-like growth factor 2 mRNA binding protein-3 (IMP3) is expressed in various cancers. In this study, we examined the diagnostic utility of IMP3 immunohistochemistry in the context of intravascular large B-cell lymphoma (IVL).

Methods: We obtained 25 skin biopsy (SB) specimens diagnosed as IVL and nine IVL-negative SB specimens from 27 IVL patients.

The Expression of Insulin-Like Growth Factor 2 Messenger RNA-Binding Protein 3 in Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma.

Langerhans cell neoplasms, which include Langerhans cell histiocytosis and Langerhans cell sarcoma, are tumors that originate from dendritic cells. Langerhans cell sarcoma is defined as a high-grade neoplasm with overtly malignant cytological features and the Langerhans cell-like phenotype, and generally has a poorer prognosis and more aggressive phenotype than Langerhans cell histiocytosis. Insulin-like growth factor 2 messenger RNA-binding protein 3 (IGF2BP3 or IMP3) is an oncofetal protein that is expressed in various cancer types; its expression is often associated with a poor prognosis and aggressive phenotype.

Successful ixazomib treatment for relapsed and refractory acute myeloid leukemia transformed from myelodysplastic syndrome.

Elevated NF-kB levels have been identified in primitive bone marrow cells from patients with MDS/AML, suggesting NF-kB as a therapeutic target in MDS/AML. We herein describe an MDS patient ineligible for SCT who, following treatment with azacitidine and bortezomib, transformed to leukemia, but maintained complete remission after monotherapy with ixazomib.

Successful Treatment of Incidental Histiocytic Sarcoma Concomitant with Laryngeal Carcinoma.

Histiocytic sarcoma (HS) is an extremely rare non-Langerhans cell disorder with an aggressive course and limited treatment options. HS most often presents at an advanced clinical stage, with a limited response to chemotherapy and high mortality. No standard treatment has been established for HS.

Neonatal Fc receptor induces intravenous immunoglobulin growth suppression in Langerhans cell histiocytosis.

The neonatal Fc receptor (FcRn) plays a role in trafficking IgG and albumin and is thought to mediate intravenous immunoglobulin (IVIG) therapy for certain diseases. IVIG can be used for the treatment of human Langerhans cell histiocytosis (LCH); however, the mechanism remains unclear. The expression and function of FcRn protein have not been studied in LCH, though the expression of FcRn messenger RNA (mRNA) have been reported.

Invasive solid papillary carcinoma with neuroendocrine differentiation of the breast: a case report and literature review.

Background: Solid papillary carcinoma (SPC) of the breast is a rare breast cancer that accounts for less than 1% of all breast cancers. The optimal clinical management of SPC remains controversial. Here, we report a case of invasive SPC with neuroendocrine differentiation in addition to review of the current literature.

The acquisition of trisomy 8 associated with Behçet's-like disease in myelodysplastic syndrome.

A relationship has been reported between myelodysplastic syndrome (MDS) and autoimmune disease. Behçet's disease is a multisystem inflammatory disorder with mucocutaneous, articular, gastrointestinal, neurological, and vascular manifestations. The co-occurrence of MDS with trisomy 8 and Behçet's-like disease was recently demonstrated.

Prediction of response to first-line therapy with ITP by flow cytometric analysis of bone marrow lymphocyte phenotypes.

In the present study, we analyzed phenotypes of cells in the lymphocyte region of bone marrow in 68 patients with primary immune thrombocytopenia (ITP) to determine whether cellular phenotype predicts response to first-line therapy (corticosteroids or corticosteroids plus intravenous immunoglobulin). In 52 newly diagnosed ITP patients, an abnormal CD4:CD8 ratio (CD4/CD8 ratio < 0.4 and 2.

Effective upfront treatment with low-dose ibrutinib for a patient with B cell prolymphocytic leukemia.

B cell prolymphocytic leukemia (B-PLL) is a rare and aggressive disease that is associated with poor survival. Although initially asymptomatic patients do not require therapy, most patients will progress and inevitably require treatment. More than 50% of patients with B-PLL carry abnormalities in the TP53 tumor suppressor gene and/or complex karyotype and show resistance to conventional chemotherapy.

Successful treatment with brentuximab vedotin for relapsed and refractory adult T cell leukemia.

Although treatments for adult T-cell leukemia/lymphoma in the past two decades have advanced, the current standard treatment for aggressive adult T-cell leukemia/lymphoma, particularly in patients who are not eligible for stem cell transplantation, remains inadequate; therefore, treatments to prolong the duration of remission and provide relevant benefits in terms of survival and quality of life are needed. Adult T-cell leukemia/lymphoma tumor cells express CD30 in some cases and the increased expression of CD30 is considered to be one of the causes of constitutive NF-κB activation in adult T-cell leukemia/lymphoma cells. Brentuximab vedotin represents a major breakthrough in the treatment of CD30-positive lymphomas.

Clinical Effect of CD25 on the Prognosis of Diffuse Large B Cell Lymphoma with Secondary Central Nervous System Relapse.

In the present study, we investigated the effects of immunophenotyping on prognosis of diffuse large B cell lymphoma (DLBCL) with central nervous system (CNS) relapse treated with rituximab-CHOP (R-CHOP). CNS relapse occurred in 9.5% of DLBCL patients.

Successful treatment with pomalidomide and intrathecal injections for central nervous system plasmacytoma in a patient under haemodialysis.

What Is Known And Objectives: The involvement of the central nervous system (CNS) in multiple myeloma (MM) is uncommon and has an extremely poor prognosis, and optimal treatment strategies for the CNS MM patients have not yet been established.

Case Summary: A 71-year-old MM patient with severe renal impairment exhibited extramedullary relapse in the CNS and progression while being treated with lenalidomide and dexamethasone. However, she achieved very good partial remission after a treatment with pomalidomide, cyclophosphamide and dexamethasone (PCD) in combination with intrathecal chemotherapy.