Paulsenehlers6179

A 57-year-old Japanese man was referred to our hospital with the chief complaint of dizziness. Our investigations showed pancytopenia that necessitated bone marrow evaluation; this evaluation revealed plasma cell proliferation that was accompanied by numerous needle-shaped crystal inclusions. Clinical and laboratory examinations were used to establish a diagnosis of multiple myeloma (MM) accompanied by Fanconi syndrome. He was administered treatment with bortezomib, lenalidomide, or thalidomide; however, he died after experiencing upper abdominal pain of unknown etiology. Histopathological examination showed needle-like inclusions in the liver and kidney and macrophages in the bone marrow, suggesting light chain deposition disease (LCDD) that could contribute to multi-organ injury. We report the rare case of a patient with needle-shaped inclusions in MM that caused LCDD.A 64-year-old woman was admitted to our hospital because of relapsed follicular lymphoma. Obinutuzumab (OBZ) and bendamustine (GB) therapy was administered for her lymphoma, and thrombocytopenia requiring platelet transfusion was observed after the first course. Although the dose of bendamustine had been reduced, her thrombocytopenia was observed again after the second course. Complete remission of her lymphoma was achieved after 4 courses of GB therapy, and the patient was switched to OBZ maintenance therapy. Nevertheless, thrombocytopenia was observed again during the maintenance therapy with OBZ alone. Observing the platelet count that changed over time after OBZ administration in detail, the platelet count started to decrease 1 hour after the end of OBZ administration, decreased to half after 6 hours, reached the lowest value 4 days after administration, and gradually recovered from 10 days after administration. Although OBZ administration-associated thrombocytopenia is a relatively common complication, acute thrombocytopenia up to 24 hours after administration is rare. However, as in this case, thrombocytopenia may progress in an extremely short time after administration, and it is necessary for clinicians to pay attention to OBZ treatment.A 69-year-old man with an unremarkable medical history presented with asymptomatic pancytopenia and diagnosed with Bence Jones protein-λ multiple myeloma (MM). Despite treatment with various chemotherapeutic regimens, myelosuppressive neutropenia occurred after each successive course; therefore, the treatment was determined to be ineffective and was discontinued. Consequently, one year after the diagnosis, a daratumumab-based therapy was initiated, and the MM was stabilized without clinical or laboratory evidences of myelosuppression. However, 18 months after the daratumumab induction, the patient developed hematochezia. Following an unremarkable lower gastrointestinal endoscopy, he presented fever and disturbed consciousness. Serum laboratory results showed liver dysfunction, and Listeria monocytogenes meningitis was diagnosed by cerebrospinal fluid examination. Empiric antibacterial treatment was administered for 3 weeks, which resolved the symptoms with no permanent neurological deficit.Daratumumab, a CD38 monoclonal antibodies, binds to expressed CD38 on myeloma cells and has an anti-myeloma cytotoxic effect but also binds to CD38 on activated macrophages. Additionally, activated macrophages play an important role in the immune defense of Listeria monocytogenes. Furthermore, inactivation of macrophages may increase the susceptibility to Listeria infection. Therefore, the possibility of infections such as Listeria meningitis should be considered in patients with MM receiving daratumumab-based therapy.Multicentric Castleman disease (MCD) comprises a heterogeneous group of lymphoproliferative disorders. Interleukin 6 (IL-6) plays an important role in the MCD pathophysiology. Here, we report the case of a 17-year-old Japanese man who presented with fever, headache, fatigue, and weight loss, with normal blood pressure. A movable mass was palpated in his lower abdomen. Laboratory tests revealed microcytic anemia and hypoalbuminemia, with elevated IL-6, sIL-2R, and vascular endothelial growth factor. Computed tomography of the abdomen demonstrated a 55-mm-diameter pelvic tumor and enlarged mesenteric lymph nodes. MCD was suspected, and the pelvic tumor resected. After the operation, his blood pressure rose slowly, and resulted to seizures of posterior reversible encephalopathy syndrome. Evaluation of hypertension revealed that plasma norepinephrine and normetanephrine concentrations were elevated, and pathological examinations showed that the resected tumor was positive for IL-6 and chromogranin-A. Therefore, we diagnosed the patient with IL-6-producing paraganglioma with MCD-mimicking symptoms. Moreover, IL-6-producing pheochromocytoma and paraganglioma should be included in differential diagnoses of MCD, even in normotensive patients.A 59-year-old woman was referred by her family doctor to our hospital owing to anemia, nausea, and malaise. She was diagnosed with primary plasma cell leukemia based on her laboratory and morphologic findings. She was treated with high dose of dexamethasone; cyclophosphamide, bortezomib, and dexamethasone; and carfilzomib, lenalidomide, and dexamethasone. She achieved partial treatment response. We switched her treatment to daratumumab, lenalidomide, and dexamethasone (DRd) owing to progression of peripheral neuropathy. Bone marrow examination performed after 15 courses of DRd revealed minimal residual disease-negative status. Sequential multidrug combination chemotherapies may be related to long-term successful disease control.Patients with HIV are at higher risk of developing thrombosis than the general population. We present a rare case of a 57-year-old Japanese man with HIV infection and a malignant lymphoma. He had fever with unknown origin and cervical lymph node swelling 2 months before his hospital visit. Because he was positive for the HIV antibody, he was referred to our HIV special outpatient section. HIV RNA level was found to be 846,680 copies/ml. Therefore, antiretroviral therapy of DTG/ABC/3TC was initiated. However, the high fever continued for 7 days after treatment initiation; moreover, renal dysfunction was progressive. After admission, antibiotic therapy was initiated, due to which the fever subsided. However, renal dysfunction continued to progress. Fourteen days later, he died due to acute renal failure with hyperkalemia. An autopsy revealed a large mass in the spleen, and histological findings revealed a diffuse large B cell lymphoma (DLBCL). Furthermore, thrombi were detected in the right and left ventricles, right atrium, iliac artery, and renal artery. Pathological findings revealed that the thrombus induced the renal failure. These thrombi contained fibrin with inflammatory cell infiltration but not tumor cells. Patients with HIV and malignant lymphoma are at a higher risk of thrombosis. It is important to consider thrombosis during the treatment of patients with HIV.A 47-year-old man was diagnosed with acute promyelocytic leukemia (APL) accompanied by pancytopenia and left forearm swelling. Complete remission was achieved with remission induction therapy using all-trans retinoic acid (ATRA), and consolidation therapy was completed. Three months after the treatment, left ear closure was observed, and a mass lesion was found in the left external auditory canal. An initial tumor biopsy only revealed inflammatory cell infiltration. Moreover, the tumor's rebiopsy performed 3 months later revealed MPO-positive and CD68-positive granulocyte infiltration. Furthermore, the rebiopsy revealed 4.9×105 copies/µgRNA of PML/RARα, the patient was diagnosed with locally recurrent APL. A bone marrow examination 2 weeks later confirmed an increase in myeloblasts and promyelocytes for the first time since the confirmation of remission. Therefore, it was diagnosed as bone marrow recurrence. Reinduction therapy using ATRA and arsenic trioxide again led to complete remission, after which autologous peripheral blood stem cell transplantation was performed. Currently, complete remission is being maintained. In this case, the recurrence of the external auditory canal lesion preceded the bone marrow recurrence. Therefore, it is important to note the nonspecific leukemia recurrence patterns of the external auditory canal.When a 74-year-old male patient visited our hospital for the treatment of herpes zoster, his computed tomography (CT) revealed a mass in his right breast, axillary lymph node enlargement, and multiple lung nodules. A histological examination of the breast and lymph node biopsies revealed diffuse large B-cell lymphoma (DLBCL) while the bronchial and salivary gland biopsies showed secondary amyloidosis and Sjögren's syndrome (SjS). According to the Ann Arbor staging, the clinical stage of the lymphoma was evaluated as IIE. The patient achieved a complete remission after six cycles of rituximab, pirarubicin, cyclophosphamide, vincristine, and prednisolone (R-THP-COP) combined with intrathecal chemotherapy to prevent meningeal infiltration and irradiation after chemotherapy. Primary breast lymphoma was diagnosed within 2% of the breast tumor. Only sixteen male cases of breast lymphoma have been previously reported. In those reports, gynecomastia and hormonal therapy accounted for nine cases, but none of the cases coexisted with SjS. The present case is suggestive of the need to investigate possible autoimmune involvement in the development of lymphoma.A 53-year-old male presented with pancytopenia for 13 months. He had a past history of follicular lymphoma and hypopharyngeal cancer, which was treated via chemotherapy and radiotherapy. Bone marrow aspiration biopsy of the patient revealed a hypocellular marrow with 32% of hypergranular blasts without Auer bodies. There were also erythroid and megakaryocytic dysplasia in the bone marrow. Although the PML/RARA transcript was detected by fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR), the G-banding karyotype analysis showed a complex karyotype without t (15;17). The PML/RARA fusion signal was identified on chromosome 15 by metaphase FISH. The patient was diagnosed of therapy-related acute promyelocytic leukemia (t-APL) with cryptic PML/RARA. He successfully attained molecular complete remission with all-trans retinoic acid (ATRA) and two courses of arsenic trioxide (ATO). He was subsequently administered nivolumab without ATRA maintenance therapy because of a progressing metastasis of a hypopharyngeal cancer to the lung. The patient had a relapse of t-APL following nine courses of nivolumab, 8 months after ending consolidation therapy with ATO. Reinduction therapy with ATRA was not effective for the relapsed t-APL that was accompanied by del (5q) and monosomy 7. Little has been previously reported on t-APL with cryptic PML/RARA. Therefore, the clinical course of this patient may provide useful insights about the characteristics of t-APL with cryptic PML/RARA.Central venous catheter (CVC) insertion is an essential medical procedure in patients with hematologic disorders. Recently, the use of peripherally inserted central venous catheters (PICCs) for CVC insertion has been increasing. However, the suitable arm for PICC insertion has not been fully discussed. This study retrospectively examined catheter-related complications on the insertion side of PICC in patients with hematologic disorders. Total 809 PICCs were inserted in 316 patients with hematologic disorders. The insertion side was the right upper arm for 515 PICCs and the left upper arm for 294 PICCs. In a direct comparison between the insertion side groups, there was no obvious difference in the basic demographic characteristics of the patients except the age at the time of insertion. Moreover, there was no significant difference in the reasons for PICC removal, and the incidence of catheter-related bloodstream infection was similar and low in both the study groups. During the selection of the insertion side for PICC, it may be possible to consider the side that seems more suitable for insertion by confirming the vessels in both upper arms with ultrasonography, and, if the conditions are equivalent, to consider the side where the patient wishes to insert the PICC.