Rare Presentation Of Multiple Myeloma Research Articles

The Importance of 18F-FDG PET/CT in a Rare Case of Multiple Myeloma Presenting With Nasal Plasmacytoma.

Nasopharyngeal plasmacytoma is a rare presentation of multiple myeloma. We emphasize the importance of imaging by presenting the 18F-FDG PET/CT findings of a patient with nasopharyngeal plasmacytoma and lytic hypermetabolic bone lesions.

Amyloid cast nephropathy: A rare presentation of multiple myeloma–associated light chain amyloidosis

Introduction: Monoclonal immunoglobulins can cause a variety of histologic patterns of kidney injury, depending on the physicochemical properties. Multiple myeloma manifests more often as light-chain nephropathy. On the other hand, light-chain amyloidosis leads to glomerular and vascular amyloid deposits, but a less common presentation with amyloid casts has also been described. Rarely, more than one histologic pattern can be present in the same patient. Case report: We report a case of a 73-year-old man, diagnosed with multiple myeloma that 8 months after achieving partial response to chemotherapy develops acute kidney injury and nephrotic syndrome. Kidney biopsy revealed features of light-chain nephropathy, amyloid cast nephropathy, and glomerular and vascular amyloid deposits. Immunofluorescence was positive for IgA (++) and lambda chains (+++) and negative for kappa chains. After the diagnosis of multiple myeloma-associated light-chain amyloidosis, chemotherapy was initiated; unfortunately, the patient died 1 month after the diagnosis. Conclusion: Amyloid casts, isolated or accompanied by other renal or extra-renal amyloid deposits, are another form of tubular toxicity caused by dysproteinemias and should be systematically screened in kidney biopsies.

Myelomatous Pleural Effusion in a Relapse Plasmablastic Myeloma

Multiple myeloma is characterised by malignant proliferation of plasma cells in bone marrow with rare involvement of peripheral blood (plasma cell leukaemia). Sometimes tissue involvement (plasmacytoma) is also seen. However, pleural effusion is very rare in myeloma patients. Pleural effusion in myeloma is usually secondary and reactive in nature. Malignant Myelomatous Pleural Effusion (MPE) is usually associated with poor prognosis. Hereby, the author report a case of a 46-year-old male who presented with groin pain with ureteric calculus. The patient was diagnosed with multiple myeloma with 42% plasma cells in the marrow. On treatment, patient went into remission, however he relapsed twice. On second relapse, the marrow examination showed plasma cells with plasmablastic morphology (24%). Plasmablastic morphology is associated with poor prognosis. The patient also developed pleural effusion. The cytospin smears of the pleural fluid showed clusters of atypical plasma cells (positive for CD38, CD138 and kappa light chain restriction). Thus, the present case report an extremely rare presentation of multiple myeloma with plasmablastic morphology and MPE.

Myelomatous Pleural Effusion: A Rare Involvement in Myeloma

Extramedullary disease (EMD) incidence is between 7% and 18% in multiple myeloma. Overall survival of patients who develop EMD is significantly shorter than that of patients without EMD. Malignant myelomatous pleural effusions (MPEs) are rarely observed, occurring in less than 1% of cases. The diagnosis of MPE was confirmed by the detection of myeloma cells in the pleural fluid using flow cytometric analyses. We present a case of a 67-year-old male patient with IgG-kappa myelom. After a few line treatment regimens, he was admitted to hospital with back pain and blurred consciousness, and pleural effusion was detected. Pleural fluid analysis showed malignant plasma cells. It is a rare presentation of multiple myeloma, but important in diagnosis. J Med Cases. 2020;11(3):55-56 doi: https://doi.org/10.14740/jmc3428

Rapidly Progressive Atrial Mass and Cardiac Tamponade: A Rare Presentation of Multiple Myeloma

Cardiac involvement in multiple myeloma is rare. We report a rapidly progressive atrial mass and plasma cell–infiltrated pericardial effusion with tamponade. Bone marrow biopsy and plasma immunoelectrophoresis confirmed multiple myeloma (Revised International Staging System Stage III). The patient died within 18 weeks of presentation, suggesting aggressive disease with poor prognosis. (Level of Difficulty: Beginner.)

MON-419 Sellar Plasmacytoma: A Commonly Misdiagnosed Sellar Mass

Background: Plasmacytoma, defined as neoplastic proliferation of a clone of plasma cells, is a rare presentation of multiple myeloma (MM). Plasmacytomas very rarely present in the sellar/parasellar region, with a reported prevalence of < 0.1%. Case: 51-year-old man with no past medical history presented with a 3-month history of severe headache, fatigue, and back pain. Brain MRI showed a large sellar mass 34 x 25 x 25 mm eroding the clivus and extending into the sphenoid sinus, with bilateral cavernous sinus invasion but no optic chiasm compression. Endocrine evaluation showed central hypogonadism and hypothyroidism: TSH 2.62 uIU/mL (0.45-4.5); free T4 0.8 ng/dL (0.82-1.77); total testosterone 229.3 ng/dL (264-916); LH 4.8 mIU/mL (1.7-8.6). Prolactin and AM cortisol levels were normal. Total protein and gamma gap were elevated. Patient endorsed 2 years of low libido and difficulty with erections. Physical exam showed no visual field deficit or cranial nerve palsies. Patient was presumptively diagnosed with an invasive non-functioning pituitary adenoma and underwent endoscopic endonasal trans-sphenoidal debulking of the mass. Intraoperatively, the mass was found to be fibrous and friable. Frozen pathology revealed a plasma cell neoplasm, and accordingly only a subtotal resection was performed. Final surgical pathology confirmed plasmacytoma with lambda light-chain restriction on immunostains. Post-op CT scan revealed multiple lytic lesions in the pelvis, spine, and bilateral hips. Oncologic evaluation confirmed the diagnosis of IgG lambda MM. Given evidence of systemic MM, patient was initiated on systemic chemotherapy within 20 days of surgery. Discussion: Sellar plasmacytoma is a diagnostic challenge, as preoperative imaging and clinical features may be indistinguishable from those of invasive pituitary adenoma. It often presents with mass effect symptoms such as headache, as seen in this case. However, this patient did not have visual disturbances or cranial nerve palsies (reported in 80% and 65% of cases, respectively). This patient's partial hypopituitarism is also atypical, as hypopituitarism is reported in 15% of cases. Misdiagnosis may potentially lead to inappropriate surgical approach and delay in therapy. Upon diagnosis, complete assessment for MM must be performed, as it is diagnosed concurrently with or during workup of plasmacytoma in 53% and 37% of cases, respectively. Conclusion: This case illustrates the importance of close attention to lab parameters such as gamma gap and total protein and to musculoskeletal symptoms in patients presenting with a sellar mass that were later found to be secondary to MM. Reference: (1) Lee J, Kulubya E, Pressman B, Mamelak A, Bannykh S, Zada G, and Cooper O. Sellar and clival plasmacytomas: case series of 5 patients with systematic review of 65 published cases. Pituitary. 2017 Jun;20(3):381-392

A case of multiple myeloma presenting with uric acid kidney stones

Kidney disease is common in patients with multiple myeloma. Patients often present with acute kidney failure most commonly the result of light chain cast nephropathy. Presentation can also be in the form of nephrotic syndrome associated with immunoglobulin light chain amyloidosis or monoclonal immunoglobulin deposition disease and tubulopathy as in acquired light chain Fanconi syndrome. We present a case of a 56-year-old male with multiple myeloma who presented with chronic kidney disease and uric acid stones. Chemical analysis of the nephrolithiasis showed it to be entirely composed of uric acid. Fractional excretion of uric acid was elevated at 35.9%. Kidney biopsy was consistent with light chain proximal tubulopathy. Bone marrow biopsy showed 30% kappa light chain–restricted plasma cells. Genetic sequencing identified the light chain to be from the Vk1-33 subgroup. The patient was treated with bortezomib, cyclophosphamide, and dexamethasone, with improvement of his kidney function. This case illustrates a rare presentation of multiple myeloma.

Multiple myeloma presenting as cutaneous leukocytoclastic vasculitis and eosinophilia disclosing a T helper type 1/T helper type 2 imbalance: a case report

BackgroundMultiple myeloma is a very heterogeneous disease comprising a number of genetic entities that differ from each other in their evolution, mode of presentation, response to therapy, and prognosis. Due to its more chronic nature and cumulative toxicities that patients develop from multiple lines of treatments, a number of symptoms are associated with multiple myeloma. However, the mechanisms responsible for the relationship between these symptoms and multiple myeloma currently remain unclear.Case presentationAn 85-year-old Japanese woman exhibited the rare presentation of multiple myeloma (immunoglobulin kappa chain type) with leukocytoclastic vasculitis and eosinophilia. The serum level of interferon-γ was decreased; however, serum levels of interleukin-4, interleukin-5, interleukin-6, interleukin-10, and tumor growth factor-β levels were elevated. She received a bortezomib, lenalidomide, and dexamethasone regimen. After one course of the treatment, the cutaneous manifestation rapidly improved and laboratory tests showed decrease of eosinophil cell count. Serum concentrations of immunoglobulin G decreased and plasma cells in bone marrow decreased. The serum level of interferon-γ was elevated and serum levels of interleukin-4, interleukin-5, interleukin-6, interleukin-10, and tumor growth factor-β decreased.ConclusionsIt is the first case of leukocytoclastic vasculitis and eosinophilia in multiple myeloma that was associated with a T helper type 1/T helper type 2 imbalance and T regulatory cells, and was successfully treated with bortezomib, lenalidomide, and dexamethasone. The present case reinforces the value of early evaluations for paraneoplastic symptoms in order to reach a diagnosis and allow for the prompt initiation of appropriate treatments and achieve successful therapeutic management.

Multiple myeloma revealed by a sphenoid plasmocytoma: case report and literature review

Plasma cell neoplasms can manifest as a solitary or multiple plasmocytomas and may be associated with or progressing to multiple myeloma (MM). Cranial and intracranial plasmocytomas revealing multiple myeloma are very rare and only few cases are reported in the literature. We report the case of a sphenoid plasmocytoma that revealed a multiple myeloma in a 56 year-old woman with 3 months history of temporal headache and diplopia. Magnetic resonance imaging (MRI) and computed tomography (CT) showed a sphenoid mass. An endoscopic sphenoidal biopsy was performed and the histopathological exams showed a plasmocytoma with a positive staining for CD138. Further biological studies confirmed the diagnosis of multiple myeloma with a monoclonal gamma peak of immunoglobulin (Ig) A. The patient started systemic chemotherapy and received decompressive radiation therapy on the sphenoidal sinus. She remained in remission for 8 months and died from renal dysfunction. Although the sphenoid plasmocytoma is a very rare presentation of multiple myeloma it should be considered for effective patient management and prognosis improvement

Secondary plasma cell leukemia in a patient with light chain multiple myeloma in post-chemotherapy remission phase

Plasma cell leukemia (PCL) is a rare presentation of multiple myeloma characterized by the presence of >20% plasma cells in peripheral blood, and an absolute plasma cell count >2 × 109 K/L. It is classified as primary and secondary with the latter representing a terminal event in 12% of patients. A case of a 78-year-old man who presented a secondary form of PCL during post-chemotherapy remission phase is discussed accompanied with a brief review.

Liver Failure: A Rare Presentation of Multiple Myeloma

Introduction: Multiple myeloma (MM) is a common hematologic malignancy that causes anemia, hypercalcemia, bone pain, and renal failure. Although multiple organ involvement is well-known in the course of MM, initial presentation with extramedullary disease, specifically hepatic involvement, is rare. Here, we report a patient presenting with liver failure, diagnosed with MM with biopsy-proven plasma cell infiltration of the liver. When hepatic involvement occurs in MM, it is usually associated with either primary amyloidosis or cholestatic jaundice. Work-up for amyloidosis in our patient was negative. Although our patient had cholestasis, labs were also remarkable for transaminitis, reflecting direct hepatocellular injury, which has been rarely reported previously. Case Description: A 56-year-old Caucasian female without previous liver disease or alcohol use presented with decreased mentation and weakness. Physical exam revealed lethargy and pallor without any focal neurologic deficit. Laboratory results included calcium 16.9, Hgb 10.3, platelets 22, AST 73, ALT 143, total bilirubin 6.3 with direct 4.3, alkaline phosphatase 183, and ammonia 72. During hospitalization, the patient became increasingly coagulopathic, with INR rising as high as 2.38. Immunofixation demonstrated free λ light chains, and a bone marrow biopsy showed plasma cell myeloma. Work-up of the liver dysfunction was negative for viral and autoimmune hepatitis, Wilson's disease, and hemochromatosis. A liver biopsy revealed atypical plasma cells consistent with plasma cell myeloma. The patient was treated with chemotherapy (bortezomib), liver function gradually returned to normal, and the patient's mental status improved to baseline. Discussion: Although hepatic plasma cell infiltration can be detected in up to 45% of people with MM at autopsy, it is rarely found in living patients. A literature review revealed three reports of clinically-evident liver impairment caused by MM at presentation. Our case demonstrates plasma cell infiltration causing cholestatic jaundice and hepatocellular injury, with a resulting rapid deterioration of hepatic function. Management is challenging in this case because of the limited number of chemotherapeutic agents that can be administered to patients with severe liver dysfunction. Liver failure caused solely by multiple myeloma should be considered in patients with diagnosis of MM and declining hepatic function. Prompt identification and treatment can lead to better patient outcomes, as demonstrated by our case.

Spontaneous tumour lysis syndrome in a case of multiple myeloma – A rare occurrence

We describe a case of a 40-year-old male patient who was found to have multiple myeloma with spontaneous tumour lysis syndrome (TLS), following a compression fracture of the L-2 vertebrae. Multiple myeloma was confirmed by bone marrow analysis and the M-band on serum protein electrophoresis. Hyperuricaemia (26.2 mg/dL), hyperkalaemia (> 7.0 mEq/L), hyperphosphatemia (16.2 mg of phosphorus/dL), normocalcemia and acute kidney injury, prior to anticancer treatment suggested spontaneous TLS. Inciting events for tumour lysis, such as chemotherapy, dehydration and exposure to steroids were absent. Patient received hydration, hypourecemic drugs and haemodialysis. This case report highlights the rare presentation of multiple myeloma with spontaneous TLS.

Extraskeletal multiple myeloma presenting with an atrial mass: a case report and a review of the literature

IntroductionExtraskeletal presentation at diagnosis or during the course of multiple myeloma is a rare event. The prognosis is usually very poor. At the moment there is no agreed gold standard for the treatment of this presentation.Case presentationA 79-year-old Caucasian woman was treated at our hospital for right atrial myeloma localization. Our patient showed the following signs and symptoms of congestive heart failure: dyspnea, hypotension, cyanosis and facial edema. Surgery was not considered feasible due to the extent of the disease. Our patient underwent external-beam radiation therapy using an intensity modulated technique, thus obtaining a persistent complete remission. Our patient has been in continuous complete local remission for 25 months since the end of radiotherapy.ConclusionThe role of radiotherapy is not defined in multiple myeloma with extraskeletal presentation. Our regimen seems to be effective in controlling the disease in this patient.This case report adds to the existing literature as it describes an unusual presentation of the disease and a new therapeutic approach to this rare presentation of multiple myeloma.

Thoracic Cord Compression by epidural Multiple Myeloma: A Rare Presentation of Multiple Myeloma

Multiple myeloma is a hematopoietic disorder and multicentric disease, with the most common localization being the spine. A 53-year-old male presented with progressive paraplegia, superficial and deep sensory disturbance below the level of T2. Spinal magnetic resonance image showed an epidural mass compressing the spinal cord at the level of T1 with intact bone structure. The patient underwent surgical posterior spinal decompression. Microscopic examination and immuno-histochemical studies confirmed the diagnosis of multiple myeloma of kappa subtype. The patient was subsequently started on steroids and chemotherapy for myeloma.Extra osseous epidural tumors causing compression myelopathy without evidence of destruction or collapse of vertebral bodies are relatively rare; to our knowledge very few cases exist in the literature.

Gingival Swelling As Rare Presentation of Multiple Myeloma: A Case Report and Review Of Literature

Gingival Swelling As Rare Presentation of Multiple Myeloma: A Case Report and Review Of Literature

Cytodiagnosis of multiple myeloma presenting as orbital involvement : A case report

BackgroundPlasma cell neoplasms represent autonomous proliferations of plasma cells and can manifest as diffuse myeloma with systemic involvement (plasma cell myeloma or multiple myeloma), monoclonal gammopathy of undetermined significance (MGUS), or as variants of plasma cell myeloma such as indolent myeloma, smoldering myeloma, osteosclerotic myeloma, plasma cell leukaemia and non-secretory myeloma. Localized neoplastic proliferation of plasma cells presents as solitary plasmacytoma of bone or extramedullary plasmacytoma. Involvement of orbit can occur as a solitary plasmacytoma, or as part of systemic involvement in multiple myeloma, the clinical outcome being significantly worse in the latter setting.Orbital involvement in multiple myeloma is very rare with less than 50 cases reported in the literature. Early cytological diagnosis of such lesions is vital for timely institution of appropriate therapy. As far as we are aware only six previous cases of cytological diagnosis of multiple myeloma involving the orbit are on record.Case presentationA 37 year old male presented with low grade fever showing evening rise, headache, diplopia and swelling in the right periorbital and temporal region. Imaging studies revealed destructive lesion of sphenoid, frontal bone and zygomatic arch with soft tissue component extending to infratemporal fossa and orbit. A fine needle aspirate from the temporal region swelling showed features of a plasmacytoma, and subsequent workup confirmed the presence of systemic disease. A final diagnosis of multiple myeloma with orbital involvement at presentation was made.ConclusionPresent case describes the extremely rare presentation of multiple myeloma with orbital involvement and highlights the utility of cytology in such lesions. Fine needle aspiration diagnosis of plasmacytoma at extramedullary sites offers an opportunity for non-invasive verification of systemic involvement, and thus plays a major role in early diagnosis and management of these patients.

Multiple Myeloma in Young Patients

The article by Blade et al 1 that appeared in the July 8, 1996, issue of theArchivesis an excellent review of the rare presentation of multiple myeloma in young patients. Blade and colleagues' discussion, however, does not mention the equally uncommon occurrence of the disease in very young patients who are infected with human immunodeficiency virus (HIV). Monoclonal gammopathy, 2,3 plasmacytoma, 4 and multiple myeloma 5,6 have been reported in HIV-infected patients younger than 30 years. Multiple myeloma in very young HIV-infected patients is also associated with multiple extramedullary plasmacytomas at unusual sites (eg, gastrointestinal tract, kidney, testicle, and lung). 6-8 The presence of Epstein-Barr virus genomes in the tumor tissue of 2 young patients with HIV infection and multiple myeloma 8,9 suggests a role for Epstein-Barr virus in the pathogenesis of myeloma in some patients with acquired immunodeficiency syndrome. A myelomaassociated paraprotein directed against the HIV-1 p24 antigen