The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
A variety of laboratory studies are used to evaluate patients for plasma cell myeloma. These tests include serum and urine protein electrophoresis, urine protein quantitation, serum and urine immunoglobulin quantitation, serum and urine Immunoelectrophoresis, radiographic skeletal survey, and bone marrow examination. Patients have monoclonal immunoglobulin in the serum and/or urine, with decreased amounts of normal serum immunoglobulins. Monoclonal proteins usually appear as a “spike” in the gamma globulin region on serum or urine protein electrophoresis. Free light chains may be found in the urine (Bence-Jones proteins) in the absence of a detectable serum monoclonal protein. Thus, in patients suspected of having plasma cell myeloma, both serum and urine should be examined for M-protein. Serum and urine Immunoelectrophoresis is one of the most sensitive methods to detect a monoclonal protein, and is used to determine the heavy chain class (IgM, IgG, IgA, IgD, or IgE) and light chain type (kappa or lambda). Because it is sensitive, it may detect a monoclonal protein in the absence of an apparent spike, for example, in patients with small amounts of monoclonal immunoglobulin or light chain myeloma. Plasma cell myelomas most commonly secrete monoclonal IgG (about 50% of cases), followed by IgA (about 20% of cases) or free light chains (about 20% of cases). Plasma cell myelomas that secrete IgD or IgE are unusual. Nonsecretory plasma cell myelomas are rare (fewer than 1% of cases).
B Cells and Humoral Immunity
Constantin A. Bona, Francisco A. Bonilla in Textbook of Immunology, 2019
This gammopathy results from proliferation in the bone marrow of IgM-secreting plasmacytoid cells. The clinical presentation is non-specific, most often generalized weakness, fatigue, and weight loss. Diagnosis is made by detecting the M component in immunoelectrophoresis. Common associated symptoms are anemia and hepatosplenomegaly. Bence-Jones proteinuria occurs in about 10% of patients. Osteolytic bone lesions are very rare. The disease is relatively indolent, and even though incidence is rarely before age 60, the mean survival after diagnosis is three to five years, and occasionally ten years or more. Death attributable to this disease is usually due to increased blood viscosity. This can lead to renal failure, hemorrhagic purpura of mucous membranes (paraproteinemia may interfere with coagulation), and occasionally congestive heart failure.
Inherited Differences in Alpha1-Antitrypsin
Stephen D. Litwin in Genetic Determinants of Pulmonary Disease, 2020
A common variant determining a lower concentration of alpha1-antitrypsin than a normal M is PiS. Homozygotes have a concentration in serum of approximately 100 mg/dl or less; MS heterozygotes have a concentration of 167 ± 37 mg/dl (see Table 3). Because of its fairly high concentration even in heterozygotes one S band can easily be distinguished cathodal to the two M bands in PiMs heterozygotes on acid starch gel electrophoresis or on isoelectric focusing. Crossed immunoelectrophoresis is usually not necessary for the identification of the S variant. However, PiV, a relatively rare variant, cannot be easily distinguished from PiS on starch gel electrophoresis; in order to differentiate these two variants agarose gel electrophoresis at pH 8.6 [105,106] should be employed. There may be another way of distinguishing these two variants that has not been explored. It has been reported [107,108] that the S-variant protein is more susceptible to heat inactivation in vitro: after 20 min at 56°C approximately 40% of the antitrypsin activity is abolished while alpha,-antitrypsin of type M retains 70% of its activity under the same conditions. This observation is of great interest in itself since it raises the possibility that the low serum concentration of S protein may be due to a faster catabolic rate.
Syphilitic meningomyelitis misdiagnosed as spinal cord tumor: Case and review
Published in The Journal of Spinal Cord Medicine, 2021
Huiqing Dong, Zheng Liu, Yunyun Duan, Dawei Li, Zhandong Qiu, Yaou Liu, Jing Huang, Chaodong Wang
Laboratory tests revealed normal hepatic, renal, thyroid function. She had negative angiotensin-converting enzyme, antinuclear antibodies, antineutrophil cytoplasmic antibodies, anticardiolipin antibodies and rheumatic factor. Serum immunoelectrophoresis and immunoglobulin studies showed no abnormalities. Serum lyme and brucella antibodies were negative. She had positive serum Treponema pallidum Hemagglutinin Test (TPHA) and rapid plasma reagin test (RPR, 1:4). CSF examination revealed 20 × 106 cells/l, 54 mg/dl protein, 37 mg/dl glucose and 112 mg/dl chloride. The cerebral spinal fluid (CSF) was positive with TPHA but negative for RPR. CSF revealed positive oligoband (OB), which indicated intrathecal IgG synthesis. CSF India ink stains were negative for cryptococcus. Bacterial growth was negative both on the blood and on the chocolate culture media Serum and CSF AQP-4 antibody was both negative.
AL amyloidosis diagnosed using anti-IGLL5 antibody: a case report
Published in Amyloid, 2019
Takayuki Hiroi, Toshiki Mushino, Ken Tanaka, Yoshiaki Furuya, Yoshikazu Hori, Takehiro Oiwa, Hiroshi Kobata, Yusuke Yamashita, Hiroki Hosoi, Shogo Murata, Akinori Nishikawa, Shinobu Tamura, Suguru Takeuchi, Masaharu Nohgawa, Taro Yamashita, Yukio Ando, Hiroyuki Hata, Takashi Sonoki
A 56-year-old man with no past medical history visited our hospital with a complaint of fatigue and weight loss over the last 6 months. No abnormalities were found in the physical examination. Complete blood count and blood chemistry also showed no abnormalities. Serum immunoelectrophoresis revealed a weak positivity of IgG-λ and IgA-λ. The serum-free LC kappa, lambda and k/λ ratio were 14.5 mg/L (3.3–19.4), 31.7 mg/L (5.7–26.3) and 0.46 (0.26–1.65), respectively. 18F-FDG-PET CT scan revealed osteolytic and sclerotic changes and FDG-avidity in the sternum and left iliac bone. The right axillary and bilateral parasternal lymph nodes were enlarged up to 17 mm with avid FDG uptake. Furthermore, a calcified tumor was observed in the tracheal branches, para-abdominal aorta, pleura and thyroid. A biopsy of the right axillary lymph node was performed. Amyloid deposition was suspected in direct fast scarlet staining, but we could not classify disease types in detail. Bone marrow aspiration of the left iliac bone showed that plasma cells were only 2.0% of all nucleated cells. There was no abnormality in the κ/λ ratio in bone marrow plasma cells as detected by flow cytometry. The axillary lymph node specimen was negative for κ, λ, transthyretin and amyloid A. However, the anti-IGLL5 antibody reacted with some part of the amyloid deposition site (Figure 1). We finally diagnosed the patient with AL amyloidosis. The patient received a triplet chemotherapy regimen (bortezomib, cyclophosphamide and dexamethasone [VCD]). After four cycles of VCD, autologous peripheral blood stem cell transplantation was performed following high-dose melphalan conditioning.
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