Epidemiological data and diagnosis pitfalls in aggressive extranodal non-Hodgkin’s lymphomas: current issues
Introduction
Non-Hodgkin's lymphoma is a group of malignant tumors that develop from hematopoietic cells located outside the medullary. They are one of the most common forms of hemoblastosis. Non-Hodgkin lymphoma develops in people of all ages. Morbidity of non-Hodgkin lymphoma increases with age reaching its highest level in people over 50 years of age.
Material and methods
A study was carried out through a narrative review of the literature in the form of a synthesis article. The article summarized and systematized various primary studies, dedicated to the epidemiological and diagnostic aspects of aggressive extranodal NHL.
Results
Aggressive extranodal NHL remains a major problem, with a fairly large increase in incidence globally. This trend is observed in several countries of the world; thus morbidity increases by 3% annually for women and by 4% for men. Globally, aggressive non-Hodgkin's lymphoma continues to affect the working-age population. Although patients with primary extranodal NHL tend to report to a medical specialist at a lower stage than those with primary ganglion disease, the number of those addressing in advanced stages continues to be increased.
Conclusions
Although diagnosing NHL does not involve great impediments, primary care physicians often detect patients in the late stages of the disease either because of delayed referral to the doctor or because of incorrect diagnosis. Despite the development of new methods of diagnosis and treatment, aggressive extranodal NHL continues to be a current problem of clinical medicine and public health, requiring increased managerial and financial efforts.
Clinical patterns and complete blood count parameters in young patients with primary myelofibrosis in the prefibrotic stage
Introduction
Primary myelofibrosis is a rare myeloproliferative neoplasm that affects 0.2-1.5 people per 100,000. As a rule, the diagnosis is confirmed after 60 years, but recently, hematologists around the world have encountered the problem of primary myelofibrosis in young people. The classic manifestations of myelofibrosis are characterized by splenomegaly, cytopenia, and bone marrow fibrosis, but in patients younger than 40 years, the diagnosis is most often made in the prefibrotic stage of the neoplasm. The aim of the paper is to identify and evaluate the clinical and hematological features of primary myelofibrosis in young patients in the prefibrotic stage.
Material and methods
A retrospective study was performed on clinical cases of primary myelofibrosis, registered at the Oncological Institute of the Republic of Moldova. The diagnosis was confirmed according to 2016 WHO criteria based on histological and molecular studies. We enrolled young patients under the age of 40 who had been diagnosed with prefibrosis in our study and analyzed them for clinical manifestations and complete blood count parameters. To optimize the analysis, all patients were divided into two groups according to their age: 18–29 and 30–40 years old.
Results
Changes in the complete blood count, manifested by thrombocytosis and leukocytosis, are the main laboratory patterns of primary myelofibrosis in young patients in the prefibrotic stage. The most relevant clinical features are splenomegaly and hepatomegaly, but no correlation between these manifestations has been found.
Conclusions
The classical clinical and hematological characteristics of primary myelofibrosis do not specify low- and intermediate-risk patients’ management in the prefibrotic stage, as compared with the other chronic myeloproliferative BCR-ABL-negative neoplasms. The proliferation type of primary myelofibrosis is characteristic for young patients with pre-fibrotic stage. According to our results, the main manifestations in the prefibrotic stage are detected in a complete blood count and comprise anemia, leucopenia, leukocytosis, and thrombocytosis.