Ethosuximide
Stanley R. Resor, Henn Kutt in The Medical Treatment of Epilepsy, 2020
Non-dose related adverse effects forcing discontinuation of therapy include skin rashes mostly nonspecific in nature but erythema multiforme, the Stevens-Johnson syndrome, and scleroderma (13). Hematologic problems usually occurred within the first 6 months. Reported blood dyscrasias include transient eosinophilia, leukopenia, thrombocytopenia, and pancytopenia. Clinical signs which may indicate a blood dyscrasia include fever, sore throat, petichiae, or intestinal bleeding. Complete blood counts should be performed monthly and periodically thereafter. Any granulopenia observed is likely to recover on reduction of the dose or discontinuation of the drug. A lupus-like syndrome has been reported in patients receiving ESM alone or in combination. Three types of reactions have been recognized: (1) development of antinuclear antibodies; (2) classic lupus-like illness with malar rash, arthritis, lymphadenopathy, pleural effusion, myocarditis, and pericarditis; autoimmune thyroiditis; and (3) a systemic immunologic disorder which includes the nephrotic syndrome (12). The lupus-like syndromes usually resolve on discontinuation of the medication. Hepatic toxicity is uncommon, though elevation of “liver” enzymes has been reported which resolved on discontinuing the medication. The relatively reduced risk of hepatic toxicity is an important positive feature of ESM therapy.
Managing Disaster and Understanding Disease and the Environment in the Early Eighteenth Century
Lori Jones in Disease and the Environment in the Medieval and Early Modern Worlds, 2022
In humoural theory, then, the cause of disease was not understood as external to the body, like a virus or a bacterium, but rather as a state of humoural imbalance. Dyscrasia could result from a number of different causes or disturbances, either internal (tied, for example, to menstruation or the onset of puberty) or external. Exposure to cold air, for instance, could cause an overabundance of black bile or phlegm, which could lead to respiratory illness (Brunton 2014, 105). Perhaps most notably, disease could result from the inhalation or absorption of miasmas through the pores.14 These were disease-causing foul odours or noxious vapours in the air that could be released from a variety of sources, including corpses, stagnant water or swamps, astrological events like the position of the stars or the arrival of a comet, or even God’s desire to punish a human population for its sins.15 Because people believed that many illnesses resulted from exposure to miasmas, treatments or regimens often emphasized the elimination of these corrupt vapours and the rebalancing of humours through practices such as bloodletting, purging via emesis (vomiting) or with laxatives or enemas, as well as the practice of prayer and/or the use of stones, talismans, minerals, and brews or concoctions. Such understandings and practices persisted in Europe, largely unchanged, for centuries.
Mechanisms of Fibril Formation and Cellular Response
Martha Skinner, John L. Berk, Lawreen H. Connors, David C. Seldin in XIth International Symposium on Amyloidosis, 2007
Case #2: 79 yo female presented with nausea and vomiting x 7 days. She had a long history of Crohn’s disease. Gastric biopsy demonstrated ulcers, granulation tissue and focal Congo red positive deposits of amyloid. Immunohistochemical typing of amyloid deposits in paraffin sections showed 3+ stain for kappa light chain, 3+ stain for amyloid P component, 1+ stain for AA protein, negative stains for lambda light chain and transthyretin. Post biopsy, isotypic underlying plasma cell dyscrasia was demonstrated. Review of the
Fluorescence in situ hybridisation combined with CD138 immunomagnetic sorting is effective to identify cytogenetic abnormalities which play significant prognostic roles in Chinese AL amyloidosis patients
Published in Amyloid, 2020
Yang Liu, Yueyun Lai, Ling Ma, Lei Wen, Wenbing Duan, Fengrong Wang, Ying Kang, Huan Chen, Yao Chen, Lu Gao, Xiaojun Huang, Jin Lu
Cytogenetic abnormalities play key prognostic roles in plasma cell dyscrasia, and their significance continues to be refined. FISH cytogenetics has been used to address chromosomal aberrations in plasma cells in AL amyloidosis and has been of great significance for obtaining outcomes that may guide therapeutic choices. Our results show that MACS can enrich sufficient plasma cells and improve the detection efficiency of cytogenetic abnormalities. For AL amyloidosis with a low proliferative index and a low bone marrow plasma cell proportion, MACS-FISH is superior to the c-FISH test. The overall abnormality rate by MACS-FISH in a Germany group was 82.9%, with t(11;14) the most prevalent aberration (59%), followed by deletion of 13q14 (29%) and amplification of 1q21 (22%) [2]. The proportion of patients with t(11;14) FISH aberration was lower in our group; however, the percentages of deletion of 13q14 and amplification of 1q21 were similar between these studies. Whether there are racial differences in the cytogenetic features of AL amyloidosis needs to be addressed in future studies. Our findings enrich what is known about the cytogenetic characteristics of this rare plasma cell disease.
Early stage IgD multiple myeloma in a 50-year-old man
Published in Baylor University Medical Center Proceedings, 2020
C. Lake Littlejohn, Andrew Whiteley, Marvin J. Stone
The orthopedic service took the patient for open reduction and internal fixation with biopsy the following day. Confusingly, intraoperative frozen section revealed only acute and chronic inflammation without evidence of malignancy. Subsequent laboratory studies showed a beta-2 microglobulin of 1.35 mg/L, albumin of 3.7 g/dL, and lactate dehydrogenase of 176 U/L. Peripheral blood flow cytometry eventually revealed a monoclonal plasma cell population making up 1% of cells analyzed. Serum and urine protein electrophoreses revealed M-spikes of 0.4 g/dL and 8.8 mg/dL, respectively. Serum immunofixation revealed a monoclonal IgD population. The lambda and kappa free light chain levels were 543.43 mg/L and 7.95 mg/L, respectively. Bone marrow biopsy was performed and revealed plasma cell dyscrasia with 20% to 25% lambda-clonal plasma cells (Figure 2). Fluorescence in situ hybridization was positive for immunoglobulin heavy chain gene rearrangement and monosomy 13. Ultimately, the right femur biopsy revealed a lambda-restricted plasma cell neoplasm.
Temporal artery involvement in AL amyloidosis: an important differential diagnosis for giant cell arteritis. A case report and literature review
Published in Modern Rheumatology Case Reports, 2020
Hiroshi Oiwa, Nagaaki Katoh, Shoichiro Kojo, Tsuneaki Yoshinaga, Kohei Taniguchi, Yasuhiro Shiote
Immunoglobulin light-chain amyloidosis, AL amyloidosis (AL), is a disorder due to extracellular tissue deposition of amyloid fibrils that are composed of fragments of monoclonal light chains. Affected patients often have underlying plasma cell dyscrasia, including multiple myeloma (MM) [1]. The organs involved include the kidney, heart, peripheral nerves, musculoskeletal systems and blood vessels. In 1986, Gertz et al. firstly reported two AL cases with temporal artery involvement, presenting with jaw claudication, myalgia and leg claudication. The authors also found that 9% of their AL cases had jaw claudication and suggested that temporal artery biopsy (TAB) with appropriate staining was necessary for the correct diagnosis [2]. Since this report, AL has been known as one of the important differential diagnoses when diagnosing giant cell arteritis (GCA). However, such cases mimicking GCA have not been reported in Japan. Herein, we report the case of an elderly female, presenting with headache and tender and enlarged temporal arteries, that was finally diagnosed with temporal artery involvement of AL amyloidosis due to Bence–Jones-type MM.
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