IgA myeloma with quadrangular crystalline inclusions

Cancer research, 1973

Myeloma plasmacytes were studied in six untreated pa tients to obtain more information on the nuclear ultrastruc tural abnormalities in these cells. Asynchrony of nucleolar, nuclear, and cytoplasmic maturation was frequently ob served in pleomorphic myeloma plasmacytes. Immature and mature myeloma plasmacytes of two patients contained intranuclear rodlets which consisted of fine filaments ar ranged in parallel. Myeloma plasmacytes in mitosis often contained persistent nucleoli, nucleolar fragments, and small nucleolus-like bodies. The mitotic division of the nu cleus was also noted in differentiated cytoplasm. In some cells in mitosis, the cytoplasm was differentiated and ap parently functional. 'This work was supported by Cancer Research Center Grant CA-I0893-P.5. 2Five of these patients died approximately 1 year after the bone mar row biopsy taken for the electron microscopy; one is surviving after 10 years.

Human Pathology, 2003

Light chain crystal deposition disease is a rare and poorly characterized entity that can be confused with a number of different conditions, depending on where the disease process is manifested. The present study explored the role of ultrastructural immunogold labeling in the diagnosis of this condition. Seven cases of light chain crystal deposition (kappa light chain-related) are reported. Immunohistochemistry and immunofluorescence techniques play a rather limited role in the evaluation of these cases, as a result of the inability to detect monoclonal kappa light chains in association with the crystalline structures or high background staining. Ultrastructural labeling is the method of choice to fully characterize these cases. How-ever, surgical pathologists must learn to recognize the findings associated with this condition to avoid misdiagnosis. If the diagnosis is at least suspected, then a complete hematologic workup may identify the underlying plasma cell dyscrasia. It must be emphasized that in some patients the plasma cell dyscrasia does not become clinically manifested until years after the diagnosis of light chain crystal deposition. HUM PATHOL 34:270-277.

Hematology & Transfusion International Journal, 2015

A 61-year-old man presented with fatigue, edema and weight loss. Bone scan demonstrated a focal lesion in the left iliac crest, which was confirmed subsequently as a lytic lesion on CT scanning. A complete blood count showed severe anemia. Hemoglobin was 72 g/L, white cell count 4.17×109/L, neutrophils 1.95×109/L, platelets 48×109/L. Serum creatinine was 328 μ mol/L; adjusted serum calcium was 2.04 mmol/L with normal serum phosphate and bicarbonate levels and anion gap. The LDH level of the patient was 208U/L, with reference range of 0-250U/L. Serum protein electrophoresis demonstrated IgG of 6.4 g/L, IgA 1.32 g/L, IgM 1.04 g/L. Urinary ß2-microglobulin protein measured 32 mg/L. Serum free κ light chains were 702 mg/dL (598-1329) and λ light chains 309 mg/dL (298-665). Bone marrow aspirate from the right superior iliac spine at the time of myeloma diagnosis showed about 15% of cells being plasma cells (Figure 1). Approximately 7% of the neoplastic plasma cells had Auer rod-like, needle-shaped or spindle-shaped intracytoplasmic inclusions (panels AD) and phagocytosed erythroid progenitors (red arrows) and mature red blood cells (blue arrows) in the aspirate (panels A-C). A smaller proportion (about 2%) of these inclusions was mixed with both needle-shaped and spindle-shaped intracytoplasmic inclusions (panels D). Unlike Auer rods in myeloid cells, these Auer rod-like inclusions were longer. Repeat bone marrow biopsy later showed persistence of these morphological findings and prominent hemophagocytosis. By flow cytometry, plasma cells expressed CD38, CD138, CD56 and CD184, and were negative for CD10, CD19, CD20, CD22, CD27 and CyclinD1, with extensive strong Kappalight chain immunostaining. Finally, the patient was diagnosed as IgG κ MM, Salmon-Durie B stage and ISS stage.

Indian Journal of Hematology and Blood Transfusion, 2008

Crystal storing histiocytosis (CSH) is a very rare association with plasma cell dyscrasias. It is presumed to be an intra-lysosomal accumulation of the secreted paraprotein aggregated into crystals and is associated with presence of variable numbers of histiocyte-like cells with phagocytosed crystalline inclusions in the bone marrow and other extramedullary sites Herein we report a case of multiple myeloma associated with CSH with a rapidly downhill clinical course. There was diagnostic confusion at the outset with a histiocytic disorder which was clarifi ed with the use of Immunohistiochemistry along with serum protein electrophoresis and immunofi xation.

American Journal of Hematology, 1990

2014

Diagnostic Cytopathology, 2012

Leukemia & lymphoma, 2017

Cancer, 1979

An unusual case of a light chain plasma cell myeloma is described. The disease was initially characterized by a diffuse lymphoplasmacytic bone marrow involvement, but subsequently developed widespread extramedullary metvtases with anaplastic tumors in the skin which histologically resembled a "histiocytic lymphoma"** Electron microscopic examination, in Vitro protein synthesis of bone marrow lymphoidal cells, chemical and immunochemical studies of serum and urine proteins, and intracellular immunoglobulin study by the immunoperoxidase technique on the skin biopsy and postmortem tumor tissue demonstrated evidence for A light chain synthesis and secretion. These findings provide further support to the notion that the wide spectrum of diverse morphologic patterns seen in lymphoplasmacytic disorders originates from the same progenitor B-lymphoid cell. Distinguishing anaplastic variant of plasma cell myeloma from other undifferentiated neoplasms offers a challenge.

Cancer, 1982

The case of a 75-year-old man with an extramedullary plasmacytoma of the stomach with peculiar histologic aspect is summarized. The cytoplasm of the proliferated cells was full of needle-shaped crystalline inclusions that made it difficult to recognize the plasma-cell nature. Under electron microscope, the crystalline inclusions were localized in the rough endoplasmic reticulum. They have a two internal line system of 75-85 A of period in a 45" angle with the main edge. The laser-ray analysis demonstrates that these two systems delineate hexagonal areas.

Journal of Clinical Pathology, 1975

results were obtained by immunofluorescence in two other cases of non-secretory myeloma (Gash, Simar, and Salmon, 1971; River, Tewksbury, and Fudenberg, 1972). Although some of these immunofluorescence studies suggest the presence of free immunoglobulin chains within the cell, they do not, as pointed out by Arend and Adamson (1974), constitute unequivocal evidence for this. We report a patient with non-secretory myeloma in whom, in addition to showing the presence of intact monoclonal immunoglobulin within the tumour cells, we have demonstrated the presence of immunoglobulin fragments as free light chains in monomeric and dimeric forms. The significance of these findings in relation to the mechanism of secretory failure is discussed.

Asian Journal of Oncology

Plasma cell myeloma (PCM) accounts for approximately 1% of malignant tumors and 10 to 15% of hematological neoplasms. It is a malignant disease characterized by abnormal proliferation of plasma cells and monoclonal immunoglobulins or free light chains (FLCs). There are many morphological variations of the myeloma plasma cells which include mature, immature, plasmablastic, and pleomorphic types. Here, we report a rare presentation of PCM with normal serum protein electrophoresis but elevated serum FLCs and having convoluted, multilobated, and monocytoid morphology. This type of morphology is related to an aggressive clinical course and resistance to conventional chemotherapy. Moreover, absence of M protein in serum/urine electrophoresis does not rule out the diagnosis of PCM and serum FLC assays plays an important role in this kind of scenarios.

https://www.ijrrjournal.com/IJRR_Vol.6_Issue.3_March2019/Abstract_IJRR0031.html, 2019

Plasma cells are known to have various nuclear and cytoplasmic inclusions. However, Auer rod-like inclusions are rarely found in these types of cells. Here, we report a case of multiple myeloma with Auer rod-like, needle-shaped intracytoplasmic inclusions in plasma cells. To the best of our knowledge, this is the first time to report such a case from Kashmir valley.

Nephrology Dialysis Transplantation, 2011

Kidney involvement with immunoglobulin crystals usually relates to a light chain of the kappa type, in MGUS or smoldering myeloma, frequently causing Fanconi's syndrome with progressive renal insufficiency. We report on a case with severe myeloma featuring lambda light chainderived crystals and acute kidney injury. Histology showed acute tubular necrosis and tubule obstruction with crystals, which were also abundant inside tubule epithelial cells, macrophages and bone marrow plasma cells. The light chain variable domain had a normal overall primary structure but included 11 somatic mutations, 3 of which likely increased the surface hydrophobicity, as observed in previously reported kappa-type crystals.

We present the case of an 80–year–old man who was admitted for anemia, back pain and progressive weakness. After a workup of clinical and laboratory data, the final diagnosis was multiple myeloma. The bone marrow aspirate revealed 53% myeloma cells with peculiar and rare morphological features: numerous large asurophilic–bright red granules – mucopolizaccharides and immunoglobulins secreted and accumulated in the endoplasmic reticulum, typically known as Russel bodies.