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Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Responses to plasmapheresis, corticosteroids, or IVIG are typically absent or minimal. More aggressive regimens including high-dose chemotherapy may be useful when plasma cell proliferation is systemic. Treatment of solitary plasmacytoma with radiation is recommended.
Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Solitary plasmacytoma may affect bone or an extramedullary site. Solitary plasmacytoma of bone (SPB) may involve any bone but is most common in the axial skeleton with symptoms due to local effects. Biopsy of the lesion shows a monoclonal population of plasma cells. A serum and/or urinary paraprotein has been reported in approximately 60% of patients but is usually at a low level. By definition the bone marrow and PET-CT scan should be normal with no evidence of marrow infiltration or other features of myeloma. Local radiotherapy is the preferred treatment of choice, but about two-thirds of patients develop MM at 10 years’ follow-up. Using flow cytometry, half of the patients showed occult BM infiltration and half of these cases progressed at 2 years.
Haematological malignancy
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Patients who present with a solitary plasma cell lesion might have a true solitary plasmacytoma as shown in Figure 17.1, but 60%–70% will eventually manifest the characteristic features of widespread multiple myeloma, particularly when it is found in a bone site.
Vitamin B6 deficiency as a cause of polyneuropathy in POEMS syndrome: rapid recovery with supplementation in two cases
Published in Hematology, 2022
Hajime Yasuda, Yoshiki Furukawa, Kenya Nishioka, Makoto Sasaki, Yutaka Tsukune, Shuichi Shirane, Nobutaka Hattori, Miki Ando, Norio Komatsu
Case 2 is a 51-year-old man presenting with polyneuropathy involving all four limbs, edema, hyperpigmentation, IgG-lambda type M-protein, and an elevated serum VEGF of 3350 pg/mL. CT scans revealed a thoracic spine tumor (T2-4) that was compressing the spinal cord parenchyma, and multiple osteosclerotic and osteolytic lesions in the thoracic spine. The thoracic spine tumor was resected to relieve spinal cord compression, and pathology revealed a plasmacytoma. Thus, multiple myeloma complicated by POEMS syndrome was diagnosed. Postoperative radiation therapy of 24Gy was administered to the original site of plasmacytoma, and three courses of VRD (bortezomib, lenalidomide, dexamethasone) therapy were administered. Although the patient’s gait disturbance improved and was able to walk with a cane after resection of the plasmacytoma, thereafter, his symptoms became fixed for approximately six months and numbness and loss of sensation especially in the lower extremities persisted. In October 2020, VB6 levels were found to be low at 3.8 ng/mL, and supplementation with 60 mg/day of PPH was initiated (Figure 1). Subsequently, the patient’s lingering symptoms of polyneuropathy suddenly started to improve, and he became cane-free and his gait almost normalized within two months after initiation of VB6 supplementation. The patient underwent two more courses of VRD therapy and proceeded to ASCT with melphalan 200 mg/m2 conditioning in December 2020. The clinical course of case 2 has been previously reported elsewhere [7].
Multiple myeloma presenting as plasmacytoma causing obstructive jaundice
Published in Baylor University Medical Center Proceedings, 2020
Ted George Achufusi, Anuj Sharma, Bishnu Sapkota
A 72-year-old white man presented to our institution with complaints of abdominal discomfort and painless jaundice. On admission, his lab work was significant for abnormal liver biochemistries with alanine transaminase 417 IU/L, aspartate aminotransferase 414 IU/L, alkaline phosphatase 1309 IU/L, total bilirubin 15.9 mg/dL, and direct bilirubin 13.4 mg/dL, with normal lipase, amylase, and white blood cell count. Physical exam revealed diffuse jaundice; however, abdominal exam did not reveal any focal findings. Computed tomography of the abdomen and pelvis was significant for a large mass adjacent to the pancreatic head along with multiple retroperitoneal and intra-abdominal nodules (Figures 1 and 2). Subsequently, the patient underwent EUS-FNA of the pancreatic lesion. Tissue biopsy was positive for plasmacytoma. Histology showed sheet-like proliferation of predominantly immature atypical plasma cells with prominent nucleoli, with scattered mature plasma cells in the background.
Plasma cell granuloma of the conjunctiva in a young female
Published in Orbit, 2021
Md. Shahid Alam, Vathsalya Vijay, Atanu Barh, Krishnakumar Subramanian
The condition should be distinguished from conjunctival plasmacytoma, which can present in a similar fashion either as an isolated disease or associated with systemic multiple myeloma. Excision biopsy is the treatment of choice for both, but the former often requires postoperative radiotherapy.10 Histopathological examination of plasmacytoma usually reveals sheets of mature plasma cells without a mixed cellular population and no fibrovascular proliferation10 whereas a plasma cell granuloma is histopathologically characterised by numerous plasma cells with dense infiltration of lymphocytes along with inclusion bodies in a fibrotic background as in our case. Normal serum immunoglobin assay also ruled out plasmacytoma in our patients.