RÉSUMÉ
Lymphoplasmacytic lymphoma (LPL) is a malignant proliferation of small lymphocytes, lymphoplasmocytoid cells and plasmocytes affecting the bone marrow, lymph nodes and spleen. Its incidence is 1/100,000 and represents 8% of all lymphomas. A total of ~5% of patients with LPL may secrete non-IgM of IgG, IgA, kappa or lambda type or be non-secretory. In the present study, a case of a 62-year-old female patient who was diagnosed with non-IgM LPL with kappa light chain monoclonal paraprotein production and normal serum immunoglobulin levels was reported. The MYD88 L265P mutation was detected by molecular genetic analysis using a sample of the bone marrow. The patient underwent initial treatment with a combination of Bendamustine-Rituximab, and later on, Ibrutinib (a Bruton kinase inhibitor) was added to the treatment protocol. The authors' aim was to describe a case of a rare type of LPL studied and cured at the University Hospital 'St. Ivan Rilski', as well as to show the methods used for its diagnosis and their applicability. The difficulty in diagnosing such rare cases of LPL which are associated with marked plasmacytic differentiation and IgA paraprotein secretion resembling plasma cell neoplasia was addressed. From the other side, the characteristic features in favor of LPL diagnosis are the immunophenotype profile of plasmocytes, as well as the presence of MYD88 L265P mutation. Finally, the methods of management and treatment of this type of lymphoma were reported, highlighting the favorable effect of the treatment with Bruton TK inhibitor (Ibrutinib).
RÉSUMÉ
INTRODUCTION: Fever of unknown origin (FUO) remains one of the most challenging clinical conditions. It demands an exhaustive diagnostic approach, considering its varied etiologies spanning infectious, autoimmune, inflammatory, and malignant causes. PATIENT CONCERNS: This report shows the journey of diagnosing a 28-year-old male who presented with persistent fever and lower-extremity weakness over 9 months. Despite seeking care at multiple hospitals, a definitive diagnosis remained elusive. DIAGNOSIS: The patient underwent a series of evaluations in various specialties, including gastroenterology, infectious diseases, rheumatology, hematology, and cardiology. Multiple tests and treatments were administered, including antiviral therapy for hepatitis B and antibiotics for suspected infections. INTERVENTIONS: After an initial misdiagnosis and unsuccessful treatments, a positron emission tomography-computed tomography scan and lymph node biopsy ultimately led to the diagnosis of peripheral T-cell lymphoma-T follicular helper type (PTCL-TFH) lymphoma. The patient was referred to the hematology clinic and initiated on CHOEP (cyclophosphamide, vincristine, etoposide, and prednisone) chemotherapy. OUTCOMES: The patient showed a positive response to CHOEP therapy, as indicated by a posttreatment positron emission tomography-computed tomography scan. He reported a significant improvement in his quality of life. Additional rounds of the same regimen were planned to further manage the lymphoma. CONCLUSION: This case emphasizes the importance of a comprehensive and persistent diagnostic approach in managing FUO. Initially, the focus on infectious causes led to extensive treatments, but the disease's progression and complications shifted attention to other specialties. The eventual diagnosis of PTCL-TFH lymphoma highlights the significance of advanced imaging techniques and multidisciplinary collaboration in uncovering elusive diagnoses. Thorough surveillance, timely reassessments, and repeated testing can uncover definitive changes critical for diagnosis. PTCL-TFH lymphoma, although rare, should be considered in the differential diagnosis of FUO, especially when initial evaluations are inconclusive.
Sujet(s)
Fièvre d'origine inconnue , Lymphome T périphérique , Mâle , Humains , Adulte , Lymphome T périphérique/complications , Lymphome T périphérique/diagnostic , Lymphome T périphérique/anatomopathologie , Fièvre d'origine inconnue/diagnostic , Fièvre d'origine inconnue/étiologie , Qualité de vie , Tomographie par émission de positons couplée à la tomodensitométrie , Lymphocytes T auxiliairesRÉSUMÉ
We explored cultural-level variables and their associations with missing data in a group of immigrants from the Former Soviet Union (FSU). Elderly hypertensive women (N = 105) completed a health survey. Prevalence of missing data and z scores were calculated to determine which survey items and measures were more likely to have missing data. Hierarchical linear regressions were performed to test whether cultural variables predicted the rate of missing data beyond individual variables. Culture variables associated with survey nonresponse and missing data were related to depression, anxiety, medication beliefs and practices, attitudes toward physicians, and cultural and behavioral identity. An interpretation of the patterns of missing data and strategies to reduce the likelihood of missing data in this population are discussed. Cultural norms likely influence patients' orientations toward their health care providers. Providers would do well to normalize difficulties with medical adherence and encourage patients to ask questions about such directives. We recommend that researchers consider the cultural appropriateness of survey items and consider alternative methods (i.e., qualitative designs) for culturally sensitive topics such as mental health and sexuality.