5-azacytidine for the treatment of massive hepatosplenomegaly in a case of myelodysplastic/myeloproliferative neoplasm-unclassifiable

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mariarita Sciumè ◽  
◽  
Alessandro Loglio ◽  
Enrico Barozzi ◽  
Giusy Ceparano ◽  
...  
Keyword(s):  
Standard Treatment ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Neoplasm ◽  
World Health ◽  
World Health Organization Classification ◽  
Treatment Algorithms ◽  
Distinct Category ◽  
The World ◽  
Health Organization ◽  
Rare Diagnosis

Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN) represent a distinct category of myeloid diseases in the World Health Organization classification, defined at diagnosis by clinical, morphologic and laboratory features which overlap both those of MDS and MPN. Within the “Overlap” MDS/MPN syndromes, MDS/MPN-Unclassifiable (MDS/MPN-U) is the least well characterized. MDS/MPN-U is a rare diagnosis, making up less than 5% of all myeloid disorders with no standard prognostic or treatment algorithms. 5-azacytidine is a standard treatment for MDS, but controversial results are available about its role for MDS/MPN-U and its effectiveness on extramedullary disease and hepatosplenomegaly. We reported the clinical management of a MDS/MPN-U patient characterized by massive hepatosplenomegaly with optimal response to 5-azacytidine.

Haematopathology of classic myeloproliferative neoplasms

2020 ◽  
pp. 45-62
Author(s):  
Hans Michael Kvasnicka ◽  
Jürgen Thiele
Keyword(s):  
Continuous Improvement ◽  
Who Classification ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
World Health ◽  
Polycythaemia Vera ◽  
The World ◽  
Health Organization ◽  
Diagnostic Importance

The classification of the World Health Organization (WHO) continues to advocate the diagnostic importance of bone marrow (BM) morphology in the diagnostic workup of myeloproliferative neoplasms (MPN). In this regard, distinctive histological BM patterns characterize specific subtypes of MPN and are the key to a meaningful clinical and molecular-defined risk stratification of patients. In this regard, the morphological denominator includes a characteristic megakaryocytic proliferation along with variable changes in the granulopoiesis and erythropoiesis. Importantly, diagnosis of MPN requires absence of relevant dysgranulopoiesis or dyserythropoiesis. In terms of clinical practice, the concept of precursor stages provides the possibility of an early intervention by appropriate therapeutic regimens that might prevent fatal complications like thrombosis and haemorrhage, especially in early stages of polycythaemia vera or in primary myelofibrosis. However, the WHO classification is not aimed to capture all biological true cases of MPN or guarantee a complete diagnostic specificity and thus might be in need of continuous improvement following clinical experience.

scholarly journals Lipomatous meningioma: A rare subtype of benign metaplastic meningiomas

2017 ◽  
Vol 08 (01) ◽  
pp. 140-142 ◽  
Author(s):  
Mehmet Onur Yüksel ◽  
Mehmet Sabri Gürbüz ◽  
Osman Tanrıverdi ◽  
Sevilay Akalp Özmen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
World Health ◽  
Histopathological Diagnosis ◽  
Resonance Imaging ◽  
Cranial Magnetic Resonance Imaging ◽  
World Health Organization Classification ◽  
The World ◽  
History Of ◽  
Health Organization

ABSTRACTLipomatous meningiomas are extremely rare subtypes of benign meningiomas and are classified as metaplastic meningioma in the World Health Organization classification. We present a 77-year-old man presented with the history of a gradually intensifying headache for the last 3 months. A right frontoparietal mass was detected on his cranial magnetic resonance imaging. The patient was operated on via a right frontoparietal craniotomy, and histopathological diagnosis was lipomatous meningioma. Distinctive characteristics of lipomatous meningiomas were discussed with special emphasis to importance of immunohistochemical examinations, particularly for its differentiation from the tumors showing similar histology though having more aggressive character.

scholarly journals Limitations of the World Health Organization classification of childhood supratentorial astrocytic tumors

Cancer ◽  
2000 ◽  
Vol 88 (6) ◽  
pp. 1477-1483 ◽  
Author(s):  
Floyd H. Gilles ◽  
William D. Brown ◽  
Alan Leviton ◽  
C. Jane Tavar� ◽  
Lester Adelman ◽  
...  
Keyword(s):  
World Health Organization ◽  
World Health ◽  
Astrocytic Tumors ◽  
World Health Organization Classification ◽  
The World ◽  
Health Organization
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