REVIEW HEMATOLOGY

POEMS Syndrome

SUMMARY

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare disorder due to an underlying plasma cell clone (PC). The syndrome can affect several organs. The diagnosis is based on the presence of mandatory criteria (polyneuropathy, monoclonal plasma cell disorder) and at least one major and one minor criteria. The therapeutic regimen is determined according to the extent of the patient’s sclerotic lesions and the presence of bone marrow involvement.

(BELG J HEMATOL 2020;11(8):381-6)

Monoclonal gammopathy of renal significance

SUMMARY

Monoclonal gammopathy of renal significance (MGRS) represents a heterogeneous group of diseases in which a monoclonal immunoglobulin, secreted by a plasma or B-cell clone with no evidence of malignancy, causes renal damage. As MGRS patients have a high risk of developing permanent kidney damage, adequate diagnosis and treatment are required to preserve kidney function. In this review, we address the definition, mechanisms and classification of MGRS, and discuss how patients with suspected MGRS should be evaluated and managed.

(BELG J HEMATOL 2020;11(8):376-80)

State of the Art I: Venous thromboembolism

The first state of the art session of the 28^th annual BSTH meeting focussed on venous thromboembolism and was moderated by Alain Gadisseur and Kristel Vandenbosch. Prof. P-YLeRoux (physician in the department of Nuclear Medicine at the Brest University Hospital, France, and senior scientist in the Thrombosis Study group of Western Brittany) opened this session with a talk on the diagnosis of acute pulmonary embolism. Following up on this presentation, prof. dr. S. Konstantinides (Professor for Clinical Trials and Medical director of the multidisciplinary centre for thrombosis and haemostasis (CTH) at the University of Mainz, Germany and professor of Cardiology at the Democritus University of Thrace, Greece), turned the attention to the clinical management of pulmonary embolism.

State of the Art II: Immunothrombosis

The second state of the art session of the 2020 annual BSTH meeting focused on Immunothrombosis. First of all, Prof Adam Cunningham (University of Birmingham, UK) addressed the association between Salmonella infection and thrombosis, after which Assistant Prof. Kimberly Martinod (Catholic University Leuven, Belgium) discussed the role of neutrophil extracellular traps at the interface of thrombosis and inflammation. In a third and final lecture of the session, Dr. Frantzeska Frantzeskaki (Attikon University Hospital, NK University of Athens, Greece) turned the attention to immunothrombosis in patients with acute respiratory distress syndrome.

Is microfilaria a transfusion transmissible disease?

SUMMARY

Due to intensive travel connections and global warming, it is possible that the vector adapts to the climatic environment of the northern half-front and that an initially tropical infectious disease becomes an emerging infectious disease in European countries. For this reason, we raise the question in the Belgian Hematology Society journal: Is microfilaria a transfusion transmissible disease? The answer is no.

(BELG J HEMATOL 2020;11(7):282-5)

Adolescents/young adults and their caregiver on the onco-haematology ward

SUMMARY

In Belgium, over 1,800 adolescents and young adults (AYAs) aged 15–39 are diagnosed annually with cancer. Of all yearly new cancer diagnoses in Belgium, AYA cancers are rare because they are rare in absolute numbers, or because they are rare examples of common cancers occurring outside of the usual age range. Leukaemia and lymphoma’s represent the most common AYA haematological cancers among the AYA population. Apart from the treatment(s) of cancer, the specific needs of young people with haematological malignancies are defined as much, or more, by their age and developmental stage as their life-threatening disease. In June 2018, an AYA interest group under the guidance of “Kom op tegen Kanker” published a blueprint for age-specific care for young people with cancer to highlight the current and future needs of AYA specific cancer care. Current healthcare professional education, training programs and healthcare settings do not address AYA-specific issues. Cure and care is currently exclusively approached from paediatric or adult care perspective. This compartmentalised approach to cancer care can result in a blind spot for AYA comprehensive age developmental cancer care for youngsters and their caregivers. Between the current paediatric and adult silos of care, there is an unmet need for comprehensive AYA cancer care. This care should focus on specific topics to support young people with haematological cancer during treatment, into survivorship care or with early integration of palliative care, providing comprehensive support for AYA patient with limited-life expectations.

(BELG J HEMATOL 2020;11(7):275-81)

Leukaemic stem cells in AML: where are we now? An update on recent findings and detection

SUMMARY

Ample evidence was provided these past decades that leukaemic stem cells (LSC) play a role in the outcome of adult and paediatric acute myeloid leukaemia (AML) patients. Although it is generally accepted that the CD34+/ CD38- compartment is most LSC-enriched, novel data have emerged illustrating a distinct biology between CD34+ and CD34- AML. In this review, we discuss the main LSC phenotypes in CD34+ and CD34- AML , as they are of utmost importance for the development of broadly applicable LSC-targeted strategies. The leukaemia-initiating capacity of these cells upon xenografting is still considered to be the gold standard for LSC detection. However, more feasible techniques have been researched to allow the implementation of LSC measurements into clinical practice. Here, we summarise the current state-of-the-art methodologies using flow cytometry and molecular detection, and emphasise their relevance in terms of prognosis and targeted drug therapy.

(BELG J HEMATOL 2020;11(6):246-52)