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A 77-year-old Japanese woman with a 21-year history of seropositive, erosive rheumatoid arthritis (RA) and a 10-year history of methotrexate (MTX) therapy was admitted with malaise and mild consciousness disturbance. Laboratory data showed hypercalcemia, acute kidney injury, normocytic anaemia, and thrombocytopenia. As we first assumed drug-induced toxicity by MTX and eldecalcitol, both were discontinued and leucovorin rescue therapy and calcitonin were administered. However, her condition continued to worsen. Serum protein electrophoresis showed only a small M-peak, immunoelectrophoresis of both the serum and urine demonstrated Bence-Jones kappa (κ) type monoclonal protein without immunoglobulin heavy chain, and bone marrow examination revealed proliferation of plasma cells. We diagnosed her with Bence-Jones κ type multiple myeloma (MM) and transferred her to the department of haematology of a higher order medical institution. Conclusively, the diagnosis of immunoglobulin (Ig) D-κ type MM, a rare variant of this disorder, was determined in accordance with serum immunofixation. Several previous studies have suggested that pre-existing RA is a risk factor for MM. Although IgD MM is characterised by its clinical severity and poor prognosis compared to other subtypes, it is often misdiagnosed or mistaken as light chain type MM, as in the present case, because of the low level of IgD M-protein, resulting in delayed diagnosis. Physicians must take MM into consideration as a differential diagnosis when inactive RA patients present with inexplicable elevated calcium, renal failure, anaemia, and bone lesion symptoms and should be aware of IgD MM to establish the correct diagnosis promptly.

Unlabelled: IgD myeloma, which is particularly severe, accounts for only 1-3% of all myeloma cases, and the kappa subtype contributes only 10-30% of IgD myelomas. We report a new case., Case-Report: A 59-year-old man was admitted for inflammatory low back pain with L5 sciatica and diffuse bone pain. The symptoms had been present for 3 months, during which he had experienced a severe decline in general health. Laboratory test abnormalities included an erythrocyte sedimentation rate of 70 mm/h, normochromic normocytic aplastic anemia, hypercalcemia (3.5 mmol/l), and serum creatinine elevation to 583 micromol/l. Tests were negative for cryoglobulinemia. Serum protein electrophoresis showed hypergammaglobulinemia but no monoclonal peak. Immunoelectrophoresis, however, detected a faint IgD kappa band in the blood and a homogeneous kappa band in the urine. Bone marrow aspirated from the sternum was found to contain 30% of malignant plasma cells. Biopsies for amyloidosis were negative. Radiographs disclosed multiple punched-out lesions with no evidence of spinal cord compression. Symptomatic treatment was given to correct the hypercalcemia, and combination chemotherapy was started., Discussion: IgD kappa myeloma is a severe variant of myeloma often associated with extraosseous lesions, renal failure, and amyloidosis. The monoclonal component is absent or faint by serum protein electrophoresis, making the diagnosis difficult. The pathogenesis is unclear and the prognosis grim.

A 67-year-old woman suffered from symmetrical polyarthralgia and multiple joint swelling simulating rheumatoid arthritis (RA). Laboratory examination showed negative results for rheumatoid factor, decreased levels of IgG, IgA, and IgM, and an increased level of IgD. Immunoelectrophoresis in her serum and urine revealed an IgD-kappa monoclonal component and Bence Jones protein (kappa), respectively. A bone marrow biopsy showed an excess of atypical plasma cells. A synovial biopsy revealed amyloid deposition composed of IgD-kappa. She was diagnosed with amyloid arthropathy (AmyA) secondary to IgD-kappa multiple myeloma. It is important to pay attention to AmyA due to multiple myeloma in patients with seronegative RA.

We refer in the present article, the first case found in our laboratory of Monoclonal gammapathy of the IgD type. A 47-year-old man presented at the emergency department with a history of malaise, lethargy, tiredness, thirstiness and obscure depositions. Clinical examination revealed a normocytic anaemia. The plasma urea was 423 mg/dl and the plasma creatinine was 15, 3 mg/dl. He was admitted to hospital with a diagnosis of acute renal failure. The later electrophoresis in serum revealed a little monoclonal band that was identified as IgD-lambda type by immunofixation electrophoresis. In urine electrophoresis was observed a beta-band. Bone marrow biopsy revealed a 20% of plasmocytic cells. Renal biopsy was compatible with myelomatose lesions. Osteolytic lesions were observed.

The presence of a serum IgD monoclonal protein (M-protein) is usually indicative of a malignant plasma cell disorder. However, one case of well-documented benign monoclonal gammopathy (BMG) of IgD type has been reported. We describe another patient with IgD monoclonal gammopathy of undetermined significance (MGUS) with long-term follow-up. In this patient an IgD lambda M-protein was found on routine serum electrophoresis. She is alive and has no evidence of multiple myeloma or amyloidosis after 8 years of follow-up. Although IgD MGUS is exceedingly rare, this case demonstrates that the presence of a serum IgD M-protein is not necessarily synonymous with a malignant plasma cell process.

In a series of 2038 paraproteinemic sera, 9 IgD paraproteins (0.44%) were found. Clinical diagnosis of all the 9 was multiple myeloma. One patient had an IgD-lambda plasmacytoma which developed into plasmocellular leukemia. Our series of IgD paraproteins consisted of 6 females and 3 males with a mean age of 58.5 years. The mean concentrations of IgD paraproteins were 6.7 +/- 4.26 g/l and the mean proteinuria was 5.1 +/- 4.76 g/24 h. Eight IgD paraproteins had light chains lambda and one had light chains kappa.

Quantitative determinations were carried out of light immunoglobulin chains by the method of ring precipitation in the serum and urine of 117 patients with plasmocytic myeloma and 16 with Waldenström's macroglobulinemia. The method was found useful for the identification of the light chain of the monoclonal proteins of IgG, and IgD classes. In the case of the lambda-type light chain disease the method is helpful in the identification of the M-component of the serum. Quantitative determination of light chains of immunoglobulins by ring precipitation is not essentially superior to other immunological and electrophoretic methods of protein analysis in monitoring treatment of cases of this myeloma.

Amyloidosis associated with plasma cell dyscrasia (AAPCD) is a relatively rare clinical entity (4% of our patients with PCD) and its early recognition and distinction from multiple myeloma (MM) may be of great therapeutic and prognostic significance. Laboratory parameters, such as concentrations of normal polyclonal Ig, Bence-Jones proteins and serum monoclonal components (MC) showed in our patients lower MC concentrations than in MM, lambda-L-chains and of gamma-H-chains predominating. Sequential skeletal X-ray studies and bone marrow morphology remain essential diagnostic procedures. Due to the lack of efficient therapeutic agents for AAPCD and the great progress achieved in recent years in the treatment of secondary amyloidosis, the immunochemical analysis of the isolated amyloid fibril as well as of the surrounding 'ground substance' should be pursued in AAPCD. Our data support previous observations, that in AAPCD the amyloid fibril subunit is an L-chain fragment predominantly derived from lambda-L-chains which originates from the same clone as the MC. The localization of an enzymatic cleavage point on the L-chain, the detection of a specific proteolytic enzyme and the identification of additional components in the amyloid substance, may further elucidate the etiopathogenesis of AAPCD.

IgD myeloma is relatively rare. We wish to report 4 new cases investigated in this laboratory during the past 18 months. Extra-osseous involvement was present in 2 patients. Total serum protein concentrations were normal in all cases, while serum paraprotein peaks were inconspicuous in 2 patients. Bence Jones proteinuria of the lambda type was present in all, while free light chains could be detected in the blood in 3 patients.

An aliquot of 24-hour urine collected from leprosy patients was concentrated and examined for the presence of albumin, transferrin, IgG, IgA, IgM, IgD, D3, kappa and lambda light chains by the gel diffusion technic using respective monospecific antisera. Urinary protein excretion profile in lepromatous leprosy patients showed that while excretion of transferrin in the urine was negligible; that of IgG molecules, a substance of higher molecular weight, was significant. It is suggested that the immunoglobulins excreted in the urine may not be plasma-derived, but extravascular in origin. They are probably synthesized in the urinary tract. In the present study, out of 25 leprosy patients, 2 female patients having severe lepra reactions developed urinary tract infections. E. coli and Klebsiella were isolated from their urine. The urinary IgG levels in those two cases were found to be the highest in the series.

A patient with a fracture of the left femur was investigated for suspected multiple myeloma. Serum total proteins, protein electrophoresis, and immunoglobulin G, A and M levels were within normal limits. Bence Jones proteinuria of the lambda type was present. Subsequent investigations revealed the presence of IgD myeloma.