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Αλέξανδρος Γ. Σφακιανάκης

Tuesday, February 23, 2021

The relationship between serum ferritin level and fibrosis and splenomegaly in myelofibrosis

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Am J Blood Res. 2020 Dec 15;10(6):345-350. eCollection 2020.

ABSTRACT

INTRODUCTION: Myelofibrosis (MF) is a disease in which the grade of bone marrow fibrosis increases in proportion to the degree of extramedullary hematopoiesis and splenomegaly. Associated with increased cytokines and inflammation, anemia deepens and an increase in serum ferritin levels is also expected. There are no studies addressing the relationship between ferritin and splenomegaly or fibrosis. In this study, the relationship between serum ferritin level and splenomegaly and bone marrow fibrosis was examined.

MATERIAL AND METHOD: The study was performed retrospectively in 46 MF cases diagnosed between 2012 and 2020. MF was divided into 3 separate subgroups: Primary myelofibrosis, secondary myelofibrosis and myeloproliferative neoplasms (MDS/MPN) with myelodsplastic syndrome.

RESULTS: Thirty (28.3%) of cases were PMF, 26 (56.5%) were SMF and 7 (15.2%) w ere MDS/MPN. There was no relation found between serum ferritin and splenomegaly in none of the cases or subgroup analysis (for PMF p: 0.564, for SMF p: 0.192, for MDS/MPN p: 0.364). There was a statistically significant relationship between serum ferritin and marrow fibrosis within the group of ages 60 years and older (p: 0.016).

DISCUSSION AND CONCLUSION: Disruption of hematopoiesis and progressive splenomegaly causes an increase in iron stores associated with an increased need for transfusion. This causes iron-related organ toxicity and bone marrow hematopoiesis disruption, leading to an increase in morbidity. We see that a significant relationship between ferritin and fibrosis has been revealed in the group aged 60 years and older. It is an unprecedented study in the literature in terms of both examining the relationship ferritin and fibrosis or splenomegaly and its results.

PMID:33489443 | PMC:PMC7811910

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Fatal hyperleukocytic T lymphoblastic lymphoma/leukemia complicated with multi-gene fusion and mutation: clinical revelation and perception

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Am J Blood Res. 2020 Dec 15;10(6):440-446. eCollection 2020.

ABSTRACT

T lymphoblastic lymphoma/leukemia (T-LBL/ALL) is a highly malignant hematological tumor common in young males. Most T-LBL/ALL patients usually initially seek medical treatment for clinical manifestations of non-hematological diseases. Presently, T-ALL chemotherapy is often used for the treatment of T-LBL/ALL internationally. With the application of high-intensity standard chemotherapy, the efficacy and prognosis of T-LBL/ALL are still not optimistic. The authors present a young male patient with facial and neck edema as the initial symptoms. This young patient of T-LBL/ALL was found to have a mediastinal mass after CT examination and he was finally diagnosed as highly malignant T-LBL/ALL. Unfortunately, after undergoing three standard courses of high-intensity chemotherapy, the young male patient eventually died of white blood cell stasis and severe infection caused by hyperleukocytosis. To this end, we find that the prognosis of T-LBL/ALL with multiple gene mutations or fusions and hyperleukocytosis, is extremely poor, and probably becomes a medical problem worthy of continuing resolution in the field of hematology and oncology.

PMID:33489453 | PMC:PMC7811898

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Effects of Ibrutinib on biophysical parameters of platelet in patients with chronic lymphocytic leukaemia

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Am J Blood Res. 2020 Dec 15;10(6):311-319. eCollection 2020.

ABSTRACT

Patients with chronic lymphocytic leukemia (CLL) treated with Ibrutinib often present hemorrhagic complications. Platelets dysfunction is well documented by aggregometry and flow cytometry, but the mechanisms by which Ibrutinib treatment influences the platelets status is yet to be evaluated. The aim of this study is to identify platelet membrane parameters in chronic lymphocytic leukemia (CLL) that could be altered by Ibrutinib administration. In this paper we propose a set of fluorescence measurements of the following parameters: membrane fluidity, resting membrane potential, and reactive oxygen species production of platelets suspensions obtained from CLL patients treated or not with Ibrutinib as markers for platelets status in this pathological situation. Platelets from CLL patients treated with Ibrutinib have higher membrane fluidity, lower resting membrane pote ntial and higher level of reactive oxygen species production compared to the untreated CLL patients. These patients are also presenting higher membrane fluidity and lower resting membrane potential compared to healthy volunteers.

PMID:33489439 | PMC:PMC7811905

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Reflux clinic: proof-of-concept of a Multidisciplinary European Clinic

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Eur Arch Otorhinolaryngol. 2021 Feb 23. doi: 10.1007/s00405-021-06705-9. Online ahead of print.

NO ABSTRACT

PMID:33620580 | DOI:10.1007/s00405-021-06705-9

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Cranial aseptic osteomyelitis associated with pyoderma gangrenosum

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Neurocirugia (Astur). 2021 Feb 2:S1130-1473(21)00001-4. doi: 10.1016/j.neucir.2020.11.007. Online ahead of print.

ABSTRACT

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the othe r one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

PMID:33546980 | DOI:10.1016/j.neucir.2020.11.007

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3-Step didactic white matter dissection of human cerebellum: Micro-neuroanatomical training

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Neurocirugia (Astur). 2021 Feb 3:S1130-1473(20)30137-8. doi: 10.1016/j.neucir.2020.11.006. Online ahead of print.

ABSTRACT

OBJECTIVES: A thorough understanding of cerebellum anatomy is essential in 4th ventricle approaches (more frequent in pediatric neurosurgery), avoiding relevant complications such as cerebellar mutism. The aim of the present work is to show the feasibility of a didactic dissection of human cerebellum focusing on cerebellar peduncles and dentate nucleus (DN), which are structures at high risk during these surgical procedures.

MATERIAL AND METHODS: The cerebellum was dissected according to the Klingler method for white matter, using standard and specific microsurgery tools. Surgical microscope magnification (x6-x40) provided by a D.F. Vasconcellos M900 was required. A Canon EOS T7 18-55 mm digital camera was used and Adobe Lightroom Classic CC and Keynote were selected as photo enhancing software. Special m ethods such as LED light endoscopic transillumination were used for photographical reasons.

RESULTS: DN dissection was successfully achieved and the relations between these nucleus and the cerebellar peduncles, inferior vermis and medullary velums were described. Through this three steps dissection guide (1. tentorial surface; 2. suboccipital surface; 3. 4th ventricle structures), the most relevant anatomical structures were shown and its implications in different 4th ventricle approaches were characterised.

CONCLUSION: 3 D perspective provided by real specimen anatomical dissection is critical for learning neuroanatomy. LED transillumination was shown as a useful technique for the 4th ventricle structures photographic documentation which improves spatial recognition. This benefit can be applied for the study of the relations between the medullary velums and the rhomboid fossa foramina, which are permeable to light. The proposed three-ste ps dissection guide helps to a better understanding of human cerebellum and to gain self-confidence, allowing safer practice for neurosurgeons in all stages of their career.

PMID:33549481 | DOI:10.1016/j.neucir.2020.11.006

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A rare central nervous system tumor of childhood with spongiform appearance on brain magnetic resonance imaging; primary diffuse leptomeningeal oligodendrogliomatosis

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Neurocirugia (Astur). 2021 Feb 4:S1130-1473(21)00006-3. doi: 10.1016/j.neucir.2020.12.004. Online ahead of print.

ABSTRACT

Primary diffuse leptomeningeal oligodendrogliomatosis is a rare fatal tumor of childhood. Symptoms usually occur when the tumor causes hydrocephalus. Brain magnetic resonance imaging (MRI) may be nearly normal in the early stages of the disease, while hydrocephalus and multiple leptomeningeal cysts with spongiform appearance may appear later on. One may consider the diagnosis when radiologic findings become apparent with multiple leptomeningeal cysts. However, failure to recognize the imaging findings due to the rarity of the disease may delay the diagnosis. Here, we report a 3.5-year-old girl who presented with ataxia and vomiting and had a diagnosis of primary diffuse leptomeningeal glioneuronal tumor with remarkable brain MRI findings as diffuse multiple tiny cystic lesions on the brain and spinal cord. She benefited f rom radiotherapy and temozolomide treatment with remission of brain MRI findings. Increasing the number of reported cases will enable the elucidation of the disease's pathogenesis and the development of treatment protocols.

PMID:33549480 | DOI:10.1016/j.neucir.2020.12.004

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