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Early Detection and Diagnostic Approach Through Automated Hematological Analysis for Plasma Cell Leukemia.

Plasma cell leukemia (PCL) is a clinically aggressive variant of multiple myeloma, characterized by a high burden of circulating plasma cells, necessitating swift and accurate diagnosis due to its poor prognosis. The conventional diagnostic criteria, including the recent recommendation by the International Myeloma Working Group (IMWG) of > 5% circulating plasma cells as positive, have evolved over time. In this context, we present a detailed case report that underscores the pivotal role of the ADVIA 2120 automated hematology counter in detecting plasma cells through cytogram analysis, along wi

AL amyloidosis presenting with isolated lumbosacral radiculoplexus neuropathy.

A 45-year-old man presented with an isolated sciatic mononeuropathy, which then evolved into a lumbosacral radiculoplexus neuropathy. His initial symptoms included lower limb pain, sensory disturbance and later weakness, without autonomic dysfunction. Neurophysiology suggested a postganglionic neuropathy. MR and ultrasound scans of the thighs showed right sciatic nerve thickening, and CSF analysis showed albuminocytological dissociation. Fluorodeoxyglucose positron emission tomography (FDG PET) was unremarkable. He then developed orthostatic symptoms and urinary disturbance, and was found to h

Discriminating between Waldenström macroglobulinemia and marginal zone lymphoma using logistic LASSO regression.

Discrimination between Waldenström macroglobulinemia (WM) and marginal zone lymphoma (MZL) of the bone marrow (BM) can be difficult due to overlap in clinical, histopathologic, and immunophenotypic characteristics. We determined which characteristics can aid in the differential diagnosis of 'gray zone' cases. We compared clinical, histopathologic, immunophenotypic, and molecular features of 222 WM and 65 MZL patients. LASSO regression was employed for variable selection. The most distinguishing clinical features of WM compared to MZL were the presence of the B-symptom weight loss and IgM parap

Solitary Plasmacytoma of the Mesentery: A Systematic Clinician's Diagnosis.

INTRODUCTION: Plasmacytoma is an uncommon plasma cell neoplasm and its localized form is solitary plasmacytoma of the bone and solitary extramedullary plasmacytoma. Solitary plasmacytoma of the mesentery is extremely rare, reported only in a handful of cases. CASE PRESENTATION: A 47-year-old man with nonspecific abdominal complains was found to have an ill-defined mass on his mesenteric root. Laparoscopic biopsy and stepwise histopathological examination revealed a mesenteric plasmacytoma, and extensive imaging and laboratory investigations led to the diagnosis of the solitary mesenteric plas

A single-center case series of eight patients with the rare plasma cell dyscrasia of acquired Fanconi syndrome secondary to monoclonal gammopathy.

Acquired Fanconi syndrome (FS) is a rare presentation of monoclonal gammopathy. We retrospectively summarized the cases of eight patients with FS secondary to monoclonal gammopathy at Peking Union Medical College Hospital (PUMCH) from January 2007 to April 2014. All patients had generalized or partial impairment of proximal renal tubular function. Six patients were diagnosed with monoclonal gammopathy of undetermined significance (MGUS), and two were diagnosed with multiple myeloma (MM). Although chemotherapy was administered to the two patients with MM and to one patient with MGUS, decreased

[Discrete papular mucinosis].

BACKGROUND: Papular mucinosis is a rare dermatological disorder characterized by papules, nodules or plaques resulting from mucin deposits in the dermis, and to a certain degree, from fibrosis, without thyroid dysfunction. CASE REPORT: A 42-year-old man consulted for symmetrical papular lesions on the face, neck and shoulders. The lesions had gradually spread over some 20 years. Laboratory findings were normal. Histopathologic examination showed diffuse mucin deposits in the superficial and middle layers of the dermis, thick bands of collagen, proliferation of fibroblasts and mild perivascula

Thromboembolism risk reduction in multiple myeloma patients treated with immunomodulatory drug combinations.

Deep vein thrombosis and its lethal complication pulmonary embolism are manifestations of venous thromboembolism (VTE), which is typically associated with cancer and recent major surgery. Certain solid tumors and hematologic malignancies impose an inherently elevated risk of VTE that is compounded by chemotherapy and other risk factors. Multiple myeloma (MM) and other plasma cell dyscrasias are thrombogenic as a consequence of their multiple hemostatic effects, including elevated interleukin-6 levels, pro-coagulant antibody formation, paraprotein interference with fibrin structure, activated p

Hypercalcemia and high serum parathyroid hormone-related protein concentration in a horse with multiple myeloma.

A 13-year-old gelding was examined because of weight loss, hyperglobulinemia, and hypercalcemia. Possible causes of hypercalcemia that were considered included renal failure, primary hyperparathyroidism, vitamin D toxicosis, and malignancy. There was no history of vitamin D ingestion, and serum creatinine and parathyroid hormone concentrations were normal, making renal failure and primary hyperparathyroidism unlikely. The hypercalcemia was suspected to be a result of malignancy, but thorough testing did not reveal any neoplastic disease. Eight months later, serum parathyroid hormone-related pr

Monocytoid B cell lymphoma associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside.

Monocytoid B cell lymphoma (MBCL) is an immunologically and morphologically well-defined low-grade lymphoma with a predilection for lymph nodes of the parotid region. We describe an association of MBCL with anti-myelin-associated glycoprotein (MAG) polyneuropathy in a 53-year-old male. The diagnosis of stage IV MBCL with nodular bone marrow infiltration, Sjögren's syndrome and sensorimotor polyneuropathy was made in October 1996. Serum immunoelectrophoresis demonstrated IgMkappa paraprotein. This was then cross-reacted with epitopes of MAG and sulphated glucuronyl paragloboside (SGPG) on myeli

[Diffuse normolipidemic plane xanthomatosis and monoclonal dysglobulinemia. A case with anti-lipoprotein activity of monoclonal immunoglobulin and hypocomplementemia].

INTRODUCTION: Diffuse normolipaemic plane xanthomatosis associated with monoclonal dysglobulinaemia has been recognized as a clinical entity for more than 30 years. Antilipoprotein activity has been reported in certain cases with monoclonal immunoglobulinaemia or hypocomplementaemia. CASE REPORT: A 64-year-old woman developed simultaneously diffuse normolipaemic plane xanthomatosis and monoclonal IgG kappa dysglobulinaemia. This paraprotein was initially associated with monoclonal gammapathy of undetermined signification (called "benign") before evolving into a myeloma. This aggravation was a

Immunophenotypic and idiotypic characterisation of the leukaemic B-cells from patients with prolymphocytic leukaemia: evidence for a selective expression of immunoglobulin variable region (IGV) gene products.

B-cell prolymphocytic leukaemia (B-PLL) is a rare chronic lymphoproliferative disease characterised by a massive splenomegaly associated with a mild or no lymphadenopathy and a high leukocyte count, mostly representing prolymphocytic features. We have studied membrane expression of certain Ig VK and VH gene products in five patients with B-PLL using a panel of monoclonal anti-subgroup and anti-cross-reactive idiotype (CRI) antibodies. Membrane expression of leukocyte-associated markers has also been investigated. The leukaemic cells from four patients expressed VKIII and VKIIIb subgroup and su

Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin.

Chronic inflammatory demyelinating polyneuropathy is an immune-mediated demyelinating peripheral neuropathy usually treated with immunosuppressants. We reviewed our experience treating 15 patients (9 men, 6 women) with intravenous immunoglobulin. Six patients were on other therapies at the time of intravenous immunoglobulin infusions (4, prednisone; 2, prednisone and azathioprine). The dose of intravenous immunoglobulin was either 0.3 or 0.4 gm/kg/day for 4 to 5 days. Transient fever occurred in 1 patient. Subjective improvement in sensory symptoms was reported by almost all patients. Objectiv

Multiple myeloma in the University Hospital: a retrospective study of biodata, clinical, laboratory and radiological profiles, 1980-1987.

A retrospective study of 37 cases of multiple myeloma admitted from 1980 to 1987 to the University Hospital Kuala Lumpur, Malaysia, was carried out to analyse the biodata, clinical presentation, laboratory and radiological profiles. The cases were selected after they had satisfied preset diagnostic criteria. The mean age was 60 years. There was no sex or ethnic preponderance. The most common symptom was bone pain. Pallor was detected in 73% of the patients. Haemoglobin was less than 120 g/L in 95%, and ESR was greater than 100 mm/hr in 70% of cases. Bone marrow and trephine biopsies were diagn

Clinical and immunologic spectrum of plasmacytoid lymphocytic lymphoma without serum monoclonal IgM.

As first suggested by Lukes & Collins (1975), and recently confirmed in the literature, the pathological lesion associated with Waldenström's macroglobulinaemia can be found in patients with serum IgG or IgA monoclonal proteins. To expand upon the clinical and immunologic spectrum of this lesion, we reviewed the records of 12 patients with identical tumour morphology, though lacking an IgM paraprotein. The median age of the patients was 62 years. In five patients the tumour secreted monoclonal IgG, while one secreted monoclonal IgA. One patient had normal serum protein electrophoresis, althoug

Monoclonal Gammopathy of Renal Significance.

Monoclonal gammopathy of renal significance (MGRS) refers to a spectrum of kidney disorders caused by monoclonal immunoglobulins produced by small, often clinically silent clones of B-cells or plasma cells that do not meet the diagnostic criteria for overt haematological malignancy. These entities are frequently overlooked, as their clinical presentation often mimics more common renal diseases and typical markers of monoclonal protein may be absent. We describe the case of a 49-year-old woman with type 2 diabetes and hypertension who presented with nephrotic-range proteinuria. Despite unremark

Type 1 Cryoglobulinemic Glomerulonephritis With IgG2-Kappa Arising From Monoclonal Gammopathy of Undetermined Significance: A Case Report and Literature Review.

This case report describes a rare presentation of Type 1 Cryoglobulinemic Glomerulonephritis due to IgG2 kappa monoclonal gammopathy in a 74-year-old man with a history of Monoclonal Gammopathy of Undetermined Significance (MGUS), classifiable under Monoclonal Gammopathy of Renal Significance (MGRS). The patient presented with acute kidney injury, hypertensive urgency, and a migratory rash. Kidney biopsy revealed glomerulitis with IgG2-kappa deposition. Pseudothrombi of IgG2-kappa paraprotein were also present within glomerular capillary and arteriolar lumens, accompanied by vasculitis. Althou

Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems.

Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has hi

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… IgM-Related Disorders/Monoclonal Gammopathy of Clinical Significance (MGCS) Certain patients exhibit clinical characteristics linked to the monoclonal IgM paraprotein, but they fail to meet the diagnostic criteria for WM. … Plasma cell dyscrasia and peripheral neuropathy: Identification of the myelin antigens that react with human paraproteins. Proc Natl Acad Sci U S A. 1981;78:7139. doi: 10.1073/pnas.78.11.7139. 135. Chassande B, Leger JM, Younes-Chennoufi AB, et al. …

From the archives of MD Anderson Cancer Center: Composite mantle cell lymphoma and lymphoplasmacytic lymphoma involving bone marrow at presentation.

Composite lymphoma, defined as two or more distinct well-defined entities involving the same anatomic site, is rare. Here we report a 79-year-old woman with composite mantle cell lymphoma (MCL) and lymphoplasmacytic lymphoma (LPL) involving bone marrow at the time of initial diagnosis. The patient presented with splenomegaly and lymphadenopathy and laboratory studies showed an elevated serum IgM level and IgM kappa paraprotein. Bone marrow evaluation showed concurrent involvement by MCL and LPL, supported by immunophenotypic studies that revealed two distinct aberrant B-cell populations. Next-

A Case of Bing-Neel Syndrome Presenting Like Giant Cell Arteritis.

A 55-year-old woman presented with new-onset headache, scalp tenderness, shoulder arthralgias, night sweats, and loss of appetite. She was diagnosed with giant cell arteritis by her primary care physician and commenced on oral corticosteroids. However, her headache, scalp tenderness, and night sweats persisted. She then developed right Horner syndrome and trigeminal hypoesthesia. Extensive blood work-up revealed mildly elevated inflammatory markers and a paraproteinemia. Subsequent bone marrow biopsy showed lymphoplasmacytic lymphoma, with 10% of hemopoiesis, and staging led to the diagnosis o

Diagnosis and management of AL amyloidosis due to B-cell non-Hodgkin lymphoma.

Immunoglobulin light chain (AL) amyloidosis may be caused by a B-cell non-Hodgkin lymphoma (NHL) rather than a plasma cell neoplasm in rare cases, which presents unique diagnostic and management considerations. NHL associated with AL will often have an IgM paraprotein; thus, this disease is termed IgM-related AL amyloidosis (IgM AL). The clinical presentation of IgM AL is more likely to involve the lungs, peripheral nerves, and soft tissue; cardiac involvement is less common. Patients with IgM AL amyloidosis should undergo a lymphoma-directed work-up including evaluation for nodal and extranod