E. F. Makhnyr’, N. Shostak, N. Inasaridze, E. V. Chernaya
{"title":"Atypical clinical picture of waldenström’s macroglobulinemia: a difficult path to diagnosis","authors":"E. F. Makhnyr’, N. Shostak, N. Inasaridze, E. V. Chernaya","doi":"10.17650/1818-8338-2019-13-1-2-80-85","DOIUrl":null,"url":null,"abstract":"Objective.To describe the difficulties of diagnosis of Waldenström macroglobulinemia in a patient with atypical clinical picture.Materials and methods.Patient K., 57 years old, came in outpatient department with complaints of unilateral increase in submandibular lymph nodes on the right, discomfort in the nasopharynx, cough without sputum, increased fatigue. During the examination she was consulted by: an infectious disease specialist, otolaryngologist, surgeon, dentist, phthisiologist, hematologist and oncologist consulted her. To confirm the diagnosis conducted diagnostic activities: dynamic assessment of laboratory parameters, examination program cancer search (including multislice computed tomography and magnetic resonance imaging of various areas), with the exception of tuberculosis, monogenically study proteins in the blood and urine tests, biopsy of the ileum, immunohistochemistry trepanobiopsy.Results.During the 4‑year examination in the patient’s blood, an M-gradient in the gamma zone was detected, monoclonal immunoglobulin М-κ 19.3 g / l, Bens-Jones-κ protein in urine (daily proteinuria 0.45 g) was detected during immunofixation. In the myelogram at the light-optical level, the number of cell elements was significantly reduced with a pronounced lymphoid proliferation of 40 %, the granulocytic series and erythropoiesis were relatively narrowed, and plasma cells 6 %. Immunomorphological picture trepanobiopsy our patient based on the data of laboratory methods of research corresponds to the defeat of the bone marrow in Waldenström’s disease-κ, secretion of M-paraprotein. An important feature that allowed to go on the right path of diagnosis was almost pathognomonic for tumor lymphoproliferation, detection of Bens-Jones protein in the urine, which was absent in the onset of the disease.Conclusion.This clinical case is interesting not only by the complexity of the diagnosis of macroglobulinemia of Waldenström in General, but also by the atypical, erased clinical disease in our patient.","PeriodicalId":82998,"journal":{"name":"The Clinician","volume":"1 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2019-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"2","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Clinician","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.17650/1818-8338-2019-13-1-2-80-85","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 2
Abstract
Objective.To describe the difficulties of diagnosis of Waldenström macroglobulinemia in a patient with atypical clinical picture.Materials and methods.Patient K., 57 years old, came in outpatient department with complaints of unilateral increase in submandibular lymph nodes on the right, discomfort in the nasopharynx, cough without sputum, increased fatigue. During the examination she was consulted by: an infectious disease specialist, otolaryngologist, surgeon, dentist, phthisiologist, hematologist and oncologist consulted her. To confirm the diagnosis conducted diagnostic activities: dynamic assessment of laboratory parameters, examination program cancer search (including multislice computed tomography and magnetic resonance imaging of various areas), with the exception of tuberculosis, monogenically study proteins in the blood and urine tests, biopsy of the ileum, immunohistochemistry trepanobiopsy.Results.During the 4‑year examination in the patient’s blood, an M-gradient in the gamma zone was detected, monoclonal immunoglobulin М-κ 19.3 g / l, Bens-Jones-κ protein in urine (daily proteinuria 0.45 g) was detected during immunofixation. In the myelogram at the light-optical level, the number of cell elements was significantly reduced with a pronounced lymphoid proliferation of 40 %, the granulocytic series and erythropoiesis were relatively narrowed, and plasma cells 6 %. Immunomorphological picture trepanobiopsy our patient based on the data of laboratory methods of research corresponds to the defeat of the bone marrow in Waldenström’s disease-κ, secretion of M-paraprotein. An important feature that allowed to go on the right path of diagnosis was almost pathognomonic for tumor lymphoproliferation, detection of Bens-Jones protein in the urine, which was absent in the onset of the disease.Conclusion.This clinical case is interesting not only by the complexity of the diagnosis of macroglobulinemia of Waldenström in General, but also by the atypical, erased clinical disease in our patient.