Myocardial amyloidosis following multiple myeloma in a 38-year-old female patient: A case report

AbstractMultiple myeloma (MM) is an immunoglobulin-producing tumor of plasma cells, which occurs commonly in the elderly. The incidence of myocardial amyloidosis with MM is extremely low and early clinical manifestations are nonspecific. The diversity of clinical manifestations and first episode symptoms often cause misdiagnosis in young patients with myocardial amyloidosis following MM. In this study, we analyzed the clinical data of a young woman with MM and impaired cardiac function combined with echocardiography, electrocardiography (ECG), laboratory data, cell Congo Red staining, and other manifestations to diagnose amyloidosis. Considering the rapid progression, short survival, and poor prognosis in most patients, a clear, definitive, and timely diagnosis is essential for the treatment of patients with MM complicated with myocardial amyloidosis.

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Primary Amyloidosis Treated with Bortezomib with a Clinical and Radiological Response.

Blood ◽

10.1182/blood.v108.11.5111.5111 ◽

2006 ◽

Vol 108 (11) ◽

pp. 5111-5111 ◽

Cited By ~ 2

Author(s):

Veena S. Fauble ◽

Ila Shah-Reddy

Keyword(s):

Multiple Myeloma ◽

Laboratory Data ◽

Phase Iii ◽

Plasma Cell Dyscrasia ◽

High Dose ◽

Rapid Progression ◽

Primary Amyloidosis ◽

Elevated Alkaline Phosphatase ◽

Diffuse Infiltration ◽

Radiological Response

Abstract INTRODUCTION: Primary amyloidosis is a plasma cell dyscrasia related to multiple myeloma. The treatment for primary amyloidosis is very similar to the treatment used for multiple myeloma. A new agent, bortezomib, has shown promising results for the treatment of multiple myeloma, but has not yet been used for the treatment of primary amyloidosis. We report a case of a patient with primary amyloidosis who was treated with bortezomib as a 2nd line agent and had significant clinical and radiological regression of disease. CASE PRESENTATION: A 56 yr old male presented with complaints of anorexia, weight loss, shortness of breath, and parathesias, and RUQ pain. Physical examination and subsequent radiologic studies showed an enlarged liver mass. Laboratory data was significant for elevated alkaline phosphatase and an IgG lambda monoclonal gammapathy in both the urine and serum. A liver biopsy revealed diffuse infiltration of hepatic parenchyma by amyloid deposits. A bone marrow examination revealed 7% plasma cells. His urine immunoelectrophoresis confirmed the presence Bence Jones proteins and a monoclonal IgG lamda spike. Additional work up revealed that he had both renal and cardiac involvement. The patient was treated with alkeran 12 mg days 1–4, thalidomide 200 mg, and dexamethasone 20 mg days 1–4, 9–12, and 17–20, with a good response initially. Seven months into treatment the patient developed a gastrointestinal bleed requiring colonoscopy. Endoscopy and biopsy findings revealed amylodosis involvement of the colon. The patient was re-treated with the same regimen, this time with a partial response, but the medications were discontinued secondary to the worsening of paresthesias and postural hypotension. 2 ½ years after his initial diagnosis, bortezomib was initiated. An excellent clinical and radiological response was seen, with a reduction in the hepatic size from 20 cm to 10 cm, improved cardiac and renal function, and disappearance of the monoclonal protein from his urine. DISCUSSION: In the past few years the advances in the treatment of primary amyloidosis has suggested that intensive chemotherapy may produce disease remission and dramatic clinical improvement in selected patients. Unfortunately, the rarity and rapid progression of this disease often delays diagnoses until multiorgan involvement limits the ability to treat. Due to the similarities in treatment between amyloidosis and myeloma, data was extrapolated from studies conducted in myeloma, and subsequently applied in this patient. A randomized phase III multicenter trial compared bortezomib with high dose dexamethasone in 669 patients with relapsed/refractory myeloma (APEX™ trial). The overall response rate (38 versus 18 percent) and complete (6 versus 0.6 percent) response rates were significantly higher in patients receiving bortezomib. Patients receiving bortezomib demonstrated a median time to progression of 6.2 months versus 3.5 months for those receiving dexamethasone. Estimated one-year survival was significantly longer for bortezomib-treated patients (80 versus 66 percent). The positive results from this trial, suggested that bortezomib might also have a beneficial role in the treatment of primary amyloidosis. This case shows a documented regression of this patient’s amyloidosis due to bortezomib treatment, further studies of bortezomib in primary amyloidosis need to be performed to validate this possibility.

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Extramedullary Plasmacytoma Diagnosed in an HIV-Positive Patient by an Unusual Clinical Presentation

Case Reports in Dentistry ◽

10.1155/2016/6305173 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5

Author(s):

Paulo de Camargo Moraes ◽

Luiz Alexandre Thomaz ◽

Victor Angelo Martins Montalli ◽

José Luiz Cintra Junqueira ◽

Camila Maria Beder Ribeiro ◽

...

Keyword(s):

Multiple Myeloma ◽

Plasma Cells ◽

Clinical Presentation ◽

Extramedullary Plasmacytoma ◽

Positive Patient ◽

Treatment Center ◽

Hiv Positive ◽

Rapid Progression ◽

Oral Diseases ◽

Infected Woman

The aim of this paper is to describe a case report of EMP in an HIV-positive patient. A 44-year-old, dark-skinned HIV-infected woman was referred to the Oral Diseases Treatment Center with a swelling at palate and left gingival fornix in the maxilla. Biopsy was taken and the oral lesion was diagnosed as EMP with well-differentiated plasma cells and restriction of the lambda light-chain. Skeletal survey was performed and no radiograph alterations were observed, thus supporting the diagnosis of EMP. Patient was referred to treatment and after two months of chemo and radiotherapy, an expanding lesion was observed in L5/S1 patient’s vertebrae. Biopsy of the spinal lesion was consistent with lymphoma with plasmocitary differentiation, supporting the diagnosis of multiple myeloma (MM). Regarding the medical history, the final diagnostic was an oral extramedullary plasmacytoma with rapid progression into multiple myeloma. It is crucial to emphasize the relevance of HIV infection as a risk factor for both aggressive clinical behavior and unusual clinical presentation of extramedullary plasmacytoma cases.

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Immunological Dysregulation in Multiple Myeloma Microenvironment

BioMed Research International ◽

10.1155/2014/198539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-10 ◽

Cited By ~ 40

Author(s):

Alessandra Romano ◽

Concetta Conticello ◽

Maide Cavalli ◽

Calogero Vetro ◽

Alessia La Fauci ◽

...

Keyword(s):

Multiple Myeloma ◽

Bone Marrow ◽

Stromal Cells ◽

Plasma Cells ◽

Clinical Manifestations ◽

Suppressor Cells ◽

Matrix Proteins ◽

Host Immune System ◽

Hematologic Disease

Multiple Myeloma (MM) is a systemic hematologic disease due to uncontrolled proliferation of monoclonal plasma cells (PC) in bone marrow (BM). Emerging in other solid and liquid cancers, the host immune system and the microenvironment have a pivotal role for PC growth, proliferation, survival, migration, and resistance to drugs and are responsible for some clinical manifestations of MM. In MM, microenvironment is represented by the cellular component of a normal bone marrow together with extracellular matrix proteins, adhesion molecules, cytokines, and growth factors produced by both stromal cells and PC themselves. All these components are able to protect PC from cytotoxic effect of chemo- and radiotherapy. This review is focused on the role of immunome to sustain MM progression, the emerging role of myeloid derived suppressor cells, and their potential clinical implications as novel therapeutic target.

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Multiple myeloma with extensive AL amyloidosis presenting as chronic diarrhoea

BMJ Case Reports ◽

10.1136/bcr-2019-232934 ◽

2020 ◽

Vol 13 (1) ◽

pp. e232934

Author(s):

William Kogler ◽

Catarina Canha ◽

Raafat Makary ◽

Reeba Omman ◽

Carmen Liliana Isache

Keyword(s):

Multiple Myeloma ◽

Renal Insufficiency ◽

Plasma Cells ◽

Chronic Diarrhoea ◽

Al Amyloidosis ◽

Lower Extremity Weakness ◽

The Past ◽

Rare Manifestation ◽

Congo Red Staining ◽

Duodenal Biopsies

We present a 52-year-old man admitted to the hospital with diarrhoea and lower extremity weakness ongoing for the past 3 months. The patient was found to have malabsorptive diarrhoea, hypoproliferative anaemia and renal insufficiency with proteinuria. Extensive workup was performed including a bone marrow biopsy with 20% plasma cells, renal and duodenal biopsies with Congo-red staining revealed amyloid deposition. The patient was diagnosed with multiple myeloma and amyloidosis with gastrointestinal, kidney and nerve involvement explaining his presentation with diarrhoea, renal insufficiency and weakness. Throughout his admission, there were incidental findings of asymptomatic hypoglycaemia (serum blood glucose <40 mg/dL), which was later found to be caused by anti-insulin monoclonal antibodies produced by the neoplastic plasma cells. This is an extremely rare manifestation of multiple myeloma with only a few cases reported in the literature.

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Optic Neuritis in the Older Chinese Population: A 5-Year Follow-Up Study

Journal of Ophthalmology ◽

10.1155/2017/3458356 ◽

2017 ◽

Vol 2017 ◽

pp. 1-7

Author(s):

Junqing Wang ◽

Huanfen Zhou ◽

Limin Qin ◽

Chunxia Peng ◽

Jie Zhao ◽

...

Keyword(s):

Optic Neuritis ◽

Vision Loss ◽

Clinical Manifestations ◽

The Elderly ◽

Chinese Patients ◽

First Episode ◽

Fiber Layer ◽

Severe Visual Loss ◽

Older Chinese

Objective. This study aims to describe the clinical manifestations and outcomes in a cohort of older Chinese patients.Method. A retrospective study of patients aged ≥ 45 years who had a first episode of optic neuritis (ON) between May 2008 and November 2012. Clinical features at onset and last follow-up were analyzed within subgroups (age 45–65 years and age ≥ 65 years).Results. 76 patients (99 eyes) were included, of which 58% were females. The mean age at presentation was 55.53 ± 8.29 years (range: 45–83 years). Vision loss was severe at presentation, with initial best corrected vision activity (BCVA) < 20/200 in 93% and final BCVA < 20/200 in 53% of patients at 5-year follow-up. Final BCVA significantly correlated with the initial BCVA and peripapillary retinal nerve fiber layer. At last follow-up, 14.5% were diagnosed with neuromyelitis optica spectrum disorder (NMOSD), 1.3% were diagnosed with multiple sclerosis (MS), 5.2% with chronic relapsing inflammatory optic neuropathy, 1.3% with infectious ON, and 19.7% with autoimmune ON. None of the elderly group (≥65 years) developed NMOSD or MS.Conclusion. Chinese patients in the age group ≥ 65 years with ON are less likely to develop NMOSD or MS. Notwithstanding, they had more severe visual loss at onset and poor recovery.

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Multiple Myeloma Course with Renal Insufficiency in Young Patient: Case Report

Blood ◽

10.1182/blood-2020-143302 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 26-26

Author(s):

Hugo Henrique de Freitas Ferreira ◽

Alessandra Suelen Jardim Silva ◽

Lenilton Silva DA Silva Júnior ◽

Gustavo Henrique de Medeiros Oliveira ◽

Maria das Graças Pereira Araujo ◽

...

Keyword(s):

Multiple Myeloma ◽

Bone Marrow ◽

Young Patient ◽

Mononuclear Cells ◽

Plasma Cells ◽

Serum Proteins ◽

Conflicts Of Interest ◽

Young Patients ◽

Treatment Strategies ◽

Malignant Neoplasm

Introduction: Multiple myeloma (MM) is a malignant neoplasm characterized by the clonal proliferation of abnormal plasma cells in the bone marrow (OM). The average age of patients diagnosed with MM is approximately 70 years, being relatively uncommon in younger individuals. Objective: To report a case of a young patient with multiple myeloma. Case Description: A 42-year-old male patient presented with continuous and progressive low back pain for 3 months, associated with adynamia, weight loss (10 kg), episodes of constipation and bleeding in the oral cavity in this period. Examinations at the first appointment revealed moderate anemia (Hb 7.4 g / dL), leukocytosis, thrombocytopenia, hypercalcemia, and altered renal function (Cr 5.9 and Ur 178), chest tomography indicating vertebral fracture in T6, T11, L2 and L4. Referred for specialized follow-up, he performed electrophoresis of serum proteins with the presence of a monoclonal peak in the gamma globulin fraction. The immunofixation test confirmed monoclonality for IgA isotype and Kappa light chain (IgA / Kappa). The myelogram showed plasmacytosis of more than 50% of mononuclear cells in the bone marrow. He developed renal failure (with dosage of creatinine of 10.1 mg/ dL. and urea of 208 mg/dL) and hypercalcemia requiring dialysis therapy on the third day of hospitalization, having undergone chemotherapy with Bortezomib, cyclophosphamide and dexamethasone. During this period, infection by the multisensitive S. aureus in catheter occurred and, despite being treated with specific antibiotic therapy, it evolved with clinical worsening and hemodynamic instability and was referred to the Intensive Care Unit, going to death after 2 days. Conclusion: Young patients with MM may study with more aggressive characteristics. Despite the use of new therapeutic agents, more effective treatment strategies need to be studied more for patients in this age group. Disclosures No relevant conflicts of interest to declare.

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Epidemiological, clinical, and laboratory findings for patients of different age groups with confirmed coronavirus disease 2019 (COVID-19) in a hospital in Saudi Arabia

PLoS ONE ◽

10.1371/journal.pone.0250955 ◽

2021 ◽

Vol 16 (4) ◽

pp. e0250955

Author(s):

Mutasim E. Ibrahim ◽

Obaid S. AL-Aklobi ◽

Mosleh M. Abomughaid ◽

Mushabab A. Al-Ghamdi

Keyword(s):

Saudi Arabia ◽

Laboratory Data ◽

Age Groups ◽

Clinical Manifestations ◽

Hemoglobin Concentration ◽

The Elderly ◽

Laboratory Findings ◽

Reactive Protein ◽

Creatine Kinase Mb ◽

D Dimer

Background Although the coronavirus disease 2019 (COVID-19) pandemic continues to rage worldwide, clinical and laboratory studies of this disease have been limited in many countries. We investigated the epidemiologic, clinical, and laboratory findings of COVID-19 infected patients to identify the effective indicators correlated with the disease. Methods A retrospective study was conducted at King Abdullah Hospital in Bisha Province, Saudi Arabia, from March 20 to June 30, 2020. Patients of different age groups were confirmed as having COVID-19 infection using a real-time polymerase chain reaction. The demographic, clinical, and laboratory data of the patients were statistically analyzed. Results Of the 132 patients, 85 were male and 47 were female, with a mean age of 50.9 years (SD±16.7). The patients were elderly (n = 29) and adults (n = 103). Of these, 54 (40.9%) had comorbidities, (25%) were admitted to the intensive care unit (ICU), and 12 (9.1%) died. On admission, the main clinical manifestations were fever (84.1%), cough (64.4%), shortness of breath (25%), chest pain (20.5%), and fatigue (18.2%). In all patients, increased neutrophils and decreased lymphocytes were observed. Patients’ lactate dehydrogenase (LDH) was elevated. C-reactive protein (CRP) was elevated in 48.5%, D-dimer in 43.2%, and the erythrocyte sedimentation rate (ESR) in 40.9% of patients. The elderly showed higher neutrophil (p = 0.011) and lower lymphocyte (p = 0.009) counts than adults. Glucose, creatine kinase-MB, LDH, bilirubin, D-dimer, and ESR were significantly higher in the elderly than in the adults. The COVID-19 death group had a higher leucocyte count (p = 0.036), and higher urea (p = 0.029) and potassium (p = 0.022) than the recovered group but had a lower hemoglobin concentration (p = 0.018). A significant association was determined between COVID-19 death and the presence of cardiovascular disease (χ2(1) = 16.297, p<0.001), hypertension (χ2(1) = 12.034, p = 0.001), renal failure (χ2(1) = 3.843, p = 0. 05), old age (t (130) = 4.9, p <0.001), and ICU admission (χ2(1) = 17.6 (1), p<0.001). Conclusions Investigating some of the laboratory and clinical parameters could help assess the disease progression, risk of mortality, and follow up patients who could progress to a fatal condition.

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Multiple Myeloma in a Young Female Presenting with Neurological Symptoms

Case Reports in Hematology ◽

10.1155/2020/1375174 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Matthew Lee ◽

Xinmin Zhang ◽

Vinh Nguyen ◽

Monique Hartley-Brown

Keyword(s):

Multiple Myeloma ◽

Plasma Cells ◽

Young Patients ◽

Young Female ◽

Neurological Symptoms ◽

Age Group ◽

Younger Age ◽

Soft Tissue Masses ◽

New Diagnosis ◽

Extramedullary Plasmacytomas

Background. Multiple myeloma is overall the 14th most common malignancy but is rarely seen in those younger than 35 years. Those in the younger age group have been shown to have a more aggressive course but reportedly have had similar responses to treatment compared to older cohorts. Extramedullary plasmacytomas are discrete soft tissue masses of neoplastic monoclonal plasma cells that often occur in patients with multiple myeloma. Case. This case entails a young female with new diagnosis of multiple myeloma and multiple extramedullary plasmacytomas presenting with neurological symptoms. She was treated with CyBorD regimen but was refractory and progressed. Treatment was changed to DCEP regimen, and daratumumab was added for primary refractory myeloma. The patient improved rapidly. Conclusion. This is a unique case of multiple myeloma in a young female that had failed first-line treatment but responded to targeted therapy of daratumumab. It highlights that multiple myeloma may present atypically in young patients. More research is needed on appropriate initial diagnosis and treatment of patients in this age group.

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Cardiac Tamponade as First Manifestation of Multiple Myeloma: a Case Report With Literature Review

10.21203/rs.3.rs-728710/v1 ◽

2021 ◽

Author(s):

Mehdi LOUKHNATI ◽

Khaoula KHALIL ◽

Fatim Ezzahra LAHLIMI ◽

Illias TAZI

Keyword(s):

Multiple Myeloma ◽

Pericardial Effusion ◽

Cardiac Tamponade ◽

Plasma Cell ◽

Plasma Cells ◽

Hematologic Malignancy ◽

Laboratory Data ◽

Rare Condition ◽

Pericardial Fluid ◽

Left Pleural Effusion

Abstract Background: Multiple myeloma is a hematologic malignancy characterized by clonal proliferation of plasma cells, mainly in bone marrow. Extramedullary disease is reported in many cases and may occur at diagnosis, at progression, or during relapse phase. Pericardial involvement is a rare condition that usually occurs with advanced-stage disease. We report a rare case of plasma cell-based pericardial effusion with cardiac tamponade as a form of presentation of multiple myeloma.Case presentation: A 76-year-old woman was admitted to the emergency department for worsening shortness of breath, on segmental examination, she had dyspnea, engorged jugulars, muffled heart sounds and lower limb edema. A Chest X-ray demonstrated cardiomegaly with a mild left pleural effusion, laboratory data showed immunoglobulin G lambda, diagnosis of plasma cell-based pericardial effusion was established by pericardial fluid cytology. The patient received systemic chemotherapy, according to Melphalan-Prednisone and thalidomid protocol. Conclusion: Multiple myeloma with cardiac effusion should be considered for differential diagnosis of patients with recurrent and unexplained pericardial effusions.

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SERIE DE CASOS: MIELOMA MÚLTIPLE DIFERENCIACIÓN CLÍNICA, DIAGNOSTICA Y TERAPÉUTICAS EN TIEMPOS DE PANDEMIA 2020.

Revista Medica Vozandes ◽

10.48018/rmv.v32.i1.s11 ◽

2021 ◽

Vol 32 (1) ◽

pp. s21-s22

Author(s):

Luis Alberto Pinza ◽

Guido Mauricio Aguirre ◽

Nancy Janeth Leines ◽

Valeria Alexandra Jara

Keyword(s):

Multiple Myeloma ◽

San Francisco ◽

Bone Pain ◽

Plasma Cells ◽

Clinical Manifestations ◽

Definitive Diagnosis ◽

Pathological Fractures ◽

Future Studies ◽

Coronary Syndrome ◽

And Performance

Introduction Multiple myeloma is a neoplasm caused by the proliferation of a clone of plasma cells that in most cases produce a monoclonal protein. Due to its decreased incidence of 4-5 cases per 100,000 inhabitants. Case description The General Hospital San Francisco Quito-Ecuador was diagnosed during this time of pandemic: three cases of Multiple Myeloma, with diverse presentation both in clinical manifestations, laboratories and radiographs, taking us to carry out a series of clinical cases with the objective of differentiating the type of clinic, diagnosis and therapy in times of pandemic in 2020. Finding different diagnoses at admission such as acute coronary syndrome, acute pancreatitis and heart failure, which were later discarded and evidenced in general bone pain, hyperamylasemia and hyperglobulinemia, with image changes both in bone punches and pathological fractures that led to the suspicion of Multiple Myeloma and performance of a medullogram for definitive diagnosis. The subspecialty treatment will run without complications. Conclusion The main characteristics are pathological fractures that can present in the form of acute or chronic pain, as well as related to other pathologies; and since the rest of the clinical manifestations mentioned in the literature were not present. It is also worth mentioning that hyperamylasemia was a factor found in our cases that future studies could be related as a prognostic factor and mortality in multiple myeloma

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