Pulmonary Langerhans Cell Histiocytosis That Progressed from a Single-system to a Multisystem Form despite Smoking Cessation.

A 36-year-old Japanese man presented with cavities and nodular shadows in the lower lobes of his lungs and osteolytic lesions in the thoracic spine. He was diagnosed with multisystem Langerhans cell histiocytosis (LCH). Three years earlier, he had been noted to have small cavities and granular lesions noted in the upper lobes of his lungs, which later improved with smoking cessation. It was likely that his single-system pulmonary LCH (PLCH) progressed to multisystem LCH despite smoking cessation. Relapse or progression may occur in cases where PLCH lesions improve after smoking cessation. Thus, close follow-up is vital.

Complete remission of pure white cell aplasia associated with thymoma after thymectomy and cyclosporine administration.

We present the case of a 63-year-old male with pure white cell aplasia (PWCA), a rare complication of thymoma, who was successfully treated with cyclosporine A (CyA) and thymectomy. The patient presented with high fever and agranulocytosis. Complete blood count revealed a white blood cell count of 0.9 × 109/L (3% neutrophils), a hemoglobin level of 15.8 g/dL, and a platelet count of 308 × 109/L. Bone marrow (BM) aspiration revealed a hypocellular marrow lacking granulocytes. Computed tomography showed a large anterior mediastinal mass, and the patient was diagnosed with PWCA associated with thymoma. Thirteen days after the initiation of CyA treatment, myeloid cells appeared in the BM, and the neutrophil count in peripheral blood started to increase on day 18. Thymectomy was performed 3 months later. Although CyA treatment was discontinued after thymectomy, complete remission has been maintained for over 4 years. In vitro colony-forming unit granulocyte-macrophage (CFU-GM) assay using the patient's serum showed severe suppression of CFU-GM colonies in the presence of the patient's serum, suggesting the presence of CFU-GM inhibitor in the patient's serum. The efficacy of the immunosuppressive therapy and the CFU-GM assay suggests the potential involvement of an immunological mechanism in patients with thymoma-associated PWCA.

Recurrence of Psoriasis Vulgaris Accompanied by Treatment with C-C Chemokine Receptor Type 4 (CCR4) Antibody (Mogamulizumab) Therapies in a Patient with Adult T cell Leukemia/ Lymphoma: Insight into Autoinflammatory Diseases.

Adult T cell leukemia / lymphoma (ATL) is one of the most aggressive hematological malignancies caused by human T-lymphotropic virus type-I (HTLV-1). Mogamulizumab is a new defucosylated humanized monoclonal antibody agent which targets C-C chemokine receptor type 4 (CCR4) expressed occasionally on the surface of ATL cells. However, adverse events such as drug eruptions have also been highlighted, at least in part, via the dysfunction of regulatory T cells (Tregs). We herein report a pronounced recurrence of systemic psoriasis vulgaris accompanied by the treatment of mogamulizumab in a patient with ATL. Pathological examinations may suggest a mechanistic link between the recurrence of autoinflammatory diseases and anti-CCR4 antibody therapies.

[Leptomeningeal infiltlation of primary CNS B-cell lymphoma diagnosed by the biopsy of cauda equina: a case report].

A 49-year-old man was admitted to our hospital with progressive gait disturbance. Our examination revealed a low grade fever, weight loss derived muscle weakness, sensory disturbance and loss of deep tendon reflex of the lower extremities. Magnetic resonance imaging (MRI) detected an abnormal intensity and gadolinium enhancement in the cauda equina. Two weeks after admission, disturbance of consciousness and bladder appeared. Cerebrospinal fluid examination showed pleocytosis, elevated protein and soluble IL-2R, but cytological examination was class II negative. We performed a cauda equina biopsy urgently and diagnosed malignant lymphoma, of a diffuse large B-cell type. We selected combined MTX-based chemoradiotherapy and his symptoms significantly improved after a month. He achieved complete remission and remains recurrence-free after 10 months post treatment although he remains with light paraparesis and sensory disturbance of the lower extremities. He has already gone back to a normal life. An examination of cauda equina biopsy led to quick diagnosis and treatment.