[Combination therapy with efgartigimod, fostamatinib, eltrombopag and prednisolone for a patient with refractory immune thrombocytopenia].

A 50-year-old man with immune thrombocytopenic purpura (ITP) was initially treated with prednisolone after 10 years of observation, but did not respond. Treatment with the thrombopoietin receptor agonist (TPO-RA) eltrombopag failed as well. After a transient partial response with fostamatinib, platelet counts decreased again, and the patient showed a severe bleeding tendency.

Bortezomib Led to Remission of Immune Thrombocytopenic Purpura in a Patient with Smoldering Multiple Myeloma.

A 79-year-old man with smoldering multiple myeloma (MM) developed sudden severe thrombocytopenia and active bleeding. Since anti-GPIIb/IIIa autoantibodies were detected on the platelet surface, thrombocytopenia due to an autoimmune etiology was diagnosed. Although neither steroids nor intravenous immunoglobulins provided improvement, bortezomib provided rapid normalization of the platelet counts with disappearance of the anti-GPIIb/IIIb autoantibodies.

Retrospective multi-center study of Adolescent and Young Adult (AYA) Multiple Myeloma in Kansai Myeloma Forum registry.

We retrospectively analyzed the clinical features and outcomes in a real-world cohort of adolescents and the young adult (AYA) patients (age between 16 and 39 years) with symptomatic multiple myeloma (MM) registered with the Kansai Myeloma Forum. 26 patients had been diagnosed as symptomatic MM out of 3284 patients. The prevalence of AYA-MM was 0.

Retrospective analysis of plasmacytoma in Kansai Myeloma Forum Registry.

We retrospectively analyzed 51 patients with solitary plasmacytoma diagnosed from October 2002 to September 2018 from a cohort of 3575 patients with plasma cell dyscrasias registered in the Kansai Myeloma Forum. Twenty-seven patients had solitary bone plasmacytoma (SBP) and 24 had extramedullary plasmacytoma (EMP), with prevalence of 0.8% and 0.

A multi-center retrospective analysis of patients with relapsed/refractory follicular lymphoma after third-line chemotherapy.

The overall outcome of patients with advanced-stage follicular lymphoma (FL) has improved significantly. However, some patients still develop multiple relapsed/refractory FL (RRFL). To address the still-limited data on this population, we performed this multi-center retrospective study.

Pretreatment serum soluble interleukin-2 receptor level predicts survival in patients with newly diagnosed follicular lymphoma.

This retrospective, multicenter observational study investigated the prognostic value of pretreatment serum soluble interleukin-2 receptor (sIL-2R) level for outcomes of newly diagnosed follicular lymphoma (FL) grade 1-3a who required treatment at diagnosis. A total of 628 patients were recorded, and 502 of these were eligible for analysis. Patients were divided into four quartiles, based on their serum sIL-2R levels as follows: Q1 (sIL-2R < 520 IU/mL), Q2 (520 ≤ sIL-2R < 1030 IU/mL), Q3 (1030 ≤ sIL-2R < 2530 IU/mL) and Q4 (sIL-2R ≥ 2530 IU/mL).

[A case of systemic AL amyloidosis diagnosed on muscle biopsy].

A 69-year-old man was admitted to our hospital with a 1-year history of progressive easy fatigability while walking. He presented with proximal muscle weakness dominant in the lower extremities, hoarseness, and mild dysphagia. Muscle pseudo-hypertrophy was observed in the gastrocnemius.

Retrospective analysis of primary plasma cell leukemia in Kansai Myeloma Forum registry.

We retrospectively analyzed twenty-six patients with primary plasma cell leukemia (pPCL) registered from May 2005 until April 2015 by the Kansai Myeloma Forum. Twenty patients received novel agents (bortezomib or lenalidomide), and their median survival of was 34 months. The median survival of patients who underwent autologous stem cell transplantation (SCT) was 40 months, those undergoing allogeneic SCT 55 months, and those undergoing both types of SCT (auto-allo) 61 months; whereas for those who did not undergo SCT it was 28 months ( = 0.

[Lymphoplasmacytic lymphoma accompanied by transformed diffuse large B-cell lymphoma with the MYD88 mutation].

The gene mutation occurring with transformation in lymphoplasmacytic lymphoma (LPL) /Waldenström macroglobulinemia (WM) has not been fully elucidated. Herein, we report a 61-year-old man with LPL/WM. In this case, the MYD88 gene mutation appeared with transformation to diffuse large B-cell lymphoma (DLBCL).

[Second primary malignancies among patients with myeloma-related-diseases in the KMF database].

The incidence of second primary malignancies (SPMs) in Japanese patients with myeloma or myeloma-related diseases was studied by using the Kansai Myeloma Forum (KMF) database registered from November 2012 to March 2015. We studied 1,571 cases. Hematologic malignancies were documented in 10 patients, and solid tumors in 36 during this period.

Pleuroparenchymal fibroelastosis after allogenic hematopoietic stem cell transplantation: important histological component of late-onset noninfectious pulmonary complication accompanied with recurrent pneumothorax.

Late-onset noninfectious pulmonary complication after allogenic hematopoietic stem cell transplantation is an important contributing factor associated with high rate morbidity and mortality. We report a case with pleuroparenchymal fibroelastosis (PPFE) occurred after allogenic bone marrow transplantation. The onset was infiltrative shadows in upper lobes, and the haziness spread gradually throughout the lungs with recurrent episodes of pneumothorax in both lungs.

[Efficacy of rituximab for TAFRO syndrome, a variant type of multicentric Castleman's disease].

A 48-year-old woman was hospitalized because of severe thrombocytopenia, leg edema, and fever. Intravenous immunoglobulin therapy was administered, but no efficacy was obtained. Her bone marrow was dry-tap, and fibrosis was found in the biopsy specimens.