Archive

Acquired Severe Factor XI Deficiency in Children: Diagnosis and Management of One Case and Literature Review

Johanna Svahn*; Angelo Claudio Molinari; Valentina Nosratian; Laura Banov

Factor XI (FXI) is a blood coagulation protease involved in the coagulation cascade, activating the Factor IX and contributing to hemostasis. Its deficiency is associated with mild to moderate bleeding manifestations that typically occur after injury, mainly in tissues containing activators of the fibrinolysis (urinary tract, oropharynx).


Diagnostic, Therapeutic and Evolutionary Aspects of Solitary Plasmacytomas in the Clinical Hematology Department of Cnts Dakar

Mohamed Keita*; Nata Dieng; Sokhna Aissatou Touré; Alioune Badara Diallo; Elimane Seydi Bousso; Serigne Mourtalla Gueye; Fatoumata Binetou Keita; Fatma Dieng; Blaise Felix Faye; Moussa Seck; Saliou Diop

Solitary plasmacytomas occur most frequently in bones, but can also be seen in soft tissues. The aim of this study was to describe the diagnostic, therapeutic and evolutionary aspects of plasmacytoma. We carried out a retrospective descriptive study from January 2017 to January 2022 in the clinical hematology department in Dakar.


Classical BCR: ABL1-Negative Myeloproliferative Neoplasms: News in the Diagnosis from the 2022 International Consensus Classification of Hematological Malignancies

Lorenzo Cirasino*; Celeste Maria Fatone

Here we report the diagnostic criteria for these diseases according to the 2022 International Consensus Classification (ICC) of Myeloid and Lymphoid Neoplasms [1-3], a major revision of the previous editions of the World Health Organization (WHO) publications on the same topic [4-12].


Systemic Cytokine Response during the Extracorporeal Circulation in Patients with Myocardial Revascularization

Milan Lazarević*; Zorica Lazarević; Mlađan Golubović; Velimir Perić; Pavle Marjanović; Vesnamarja-nović; Saša Živić; Isidora Milić; Aleksandar Kamenov; Vladimir Stojiljković

Revascularization of the myocardium under conditions of extracorporeal circulation cause clinical problems related to the effects of the intervention itself on the immune system and inflammatory response, especially systemic inflammatory response (Engl. Systemic Inflammatory Response Syndrome, SIRS)


Infection-Related Morbidity and Mortality among Multiple Myeloma Patients in West Africa

Moussa Seck*; Moussa Traoré; Elimane Seydi Bousso; Alioune Badara Diallo; Sokhna Aissatou Touré; Mohamed Keita; Sérigne Mourtalla Guéye; Blaise Félix Faye; Abibatou Sall; Awa Oumar Touré; Saliou Diop

Multiple Myeloma (MM) is characterized by immunosuppression leading to increased susceptibility to infections. The aim of this study was to evaluate the morbidity and mortality of infections in patients followed for MM.


Second Primary Malignancies in Patients with Gastrointestinal Stromal Tumours: Ten-Year Experience from the Ottawa Hospital

Abdulhameed Alfagih; Abdulaziz AlJassim; Bader Alshamsan; Nasser Alqahtani; Timothy Asmis*

The secondary malignancies in patients with GIST are relatively high. We present our 10-year experience of SPM in patients with GIST from a regional Cancer Centre in Canada. A retrospective cohort study was performed on all GIST patients treated at TOH between January 2011 and December 2021.


Quantitative and Qualitative Changes in the Ca2+, ATP-Adenosine Cycle and ROS Metabolisms Depending On the Strength of Danger Signals during Apoptosis and Necrosis Compared to Homeostasis in Various Danger/Stress Situations

Sándor Sipka*; Kitti Pázmándi; Sándor Sipka JR; Geza Bruckner; Zsolt Bodnár

In earlier publications there are only separately mentioned that the danger/stress signals induce increased intracellular Ca2+ levels and concomitant release of adenosine triphosphate (ATP) accompanied with the production of reactive oxygen species (ROS). In such situations extracellular ATP acting on purinergic receptors type 2 (P2) can trigger the release of numerous mediator molecules helping the defence of cells...


Severe Hyperammonaemic Encephalopathy in a Patient with Secondary Plasma Cell Leukemia Treated with Daratumumab: A Case Report

Mauro Passucci*; Francesca Fazio; Maria Teresa Petrucci

Despite an improvement in prognosis due to greater availability of novel agents, such us proteasome inhibitors, immunomodulatory drugs and anti-CD38 monoclonal antibodies, Multiple Myeloma (MM) remains an incurable disease and is associated with a more aggressive and resistant course in 20-30% of cases. Disease symptoms are related to the bone marrow clonal plasma cells infiltration and MM patients presents with a variety of disease-related complications; the most common include hypercalcemia, renal insufficiency, anemia and osteolytic bone lesions...


High Curative Potential of Ibrutinib in Hairy Cell Leukemia Variant Refractory to Conventional Chemotherapy: A Case Report

Mauro Passucci; Silvio Ligia; Ilaria Del Giudice; Gianna Maria D’Elia; Giorgia Annechini; Roberta Soscia; Enrico Tiacci; Alessandro Pulsoni*

Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder with a high sensitivity to purine nucleoside analogues (PNAs). Its uncommon variant form (HCLv) has a more aggressive clinical course and an intrinsic resistance to chemotherapy. Target therapies like Bruton tyrosine kinase inhibitors (BTKi) are under investigation. We present a case of a 62-years-old female with HCLv presenting with splenomegaly and hyperleukocytosis...


Sickle Cell Disease and Transglutaminase 2

Sándor Sipka

Background: Despite the great progress in the therapy of “Sickle Cell Disease” (SCD) there are still unknown mechanisms in the pathophysiology of the disease. The recognition of these hidden pathways is worth discovering in order to apply new treatments. In this letter, I am going to draw attention to my earlier not published observations on the increased activity of Transglutaminase 2 (TG2) in the erythrocytes of patients with SCD. This approach is still new.


The Impact Of COVID-19 on the Number of In-Patient Visit in Pediatric Haemato-Oncology Ward, Ulin Hospital Banjarmasin, 2019-2021

Wulandewi Marhaeni*

Background: The Indonesian government implemented a social restriction policy which concludes large-scale social restrictions in dealing with COVID-19. This situation made a huge impact on people’s lives, especially those who need regular medication.


Steroid-Refractory Evans Syndrome Secondary to Chronic Lymphocytic Leukemia (CLL), Successfully Treated with Rituximab

Sindhusha Veeraballi; Zaineb Khawar; Brooke Kania*; Leena Bondili; Vinod Kumar; Michael Maroules

    Evans Syndrome (ES) is an autoimmune disorder where patients develop Autoimmune Hemolytic Anemia (AIHA) as well as Immune Thrombocytopenia (ITP), with or without immune neutropenia. The previous use of corticosteroid treatment for ES secondary to CLL has shown to be a temporary solution, with most patients developing recurrent disease within 36 months...


A Rare Case of Primary Epidural Cavernous Hemangioma Mimicking Lobulated Upper Lobe Lung Mass

Sindhusha Veeraballi; Nooren Mirza; Brooke Kania; Leena Bondili; Vinod Kumar*; Michael Maroules

    Isolated primary epidural hemangioma is extremely rare and constitutes approximately four percent of epidural lesions, with the cavernous subtype being the most common. Hemangiomas are benign proliferative vascular lesions that are classified based on the predominant type of vascular channels involved into capillary, cavernous, arteriovenous, or venous. These vascular lesions can also be classified into malformations that are high flow (arteriovenous) and low flow (capillary, cavernous, venous)...


Pulse Monthly Cyclophosphamide Therapy in Refractory Autoimmune Hemolytic Anemia: A New Frame Of Mind

Richmond Ronald Gomes

    High-dose cyclophosphamide, without stem cell rescue, has been used successfully to treat aplastic anemia and other autoimmune disorders. Treatment of steroid refractory Autoimmune Hemolytic Anemia (AIHA) is challenging especially with no evidence based consensus guidelines and limited resources. To determine the safety and efficacy of high dose pulse cyclophosphamide-1 g/month for four consecutive months-in our patient with severe refractory AIHA secondary to adult onset stills disease...


"Breakfasting: Breaking Vessels and Fasting Can Battle Obesity Mediated Metastatic Disease"

Gabriela D'Amico; Harini Vijayenthiran*

    Current antiangiogenic and immunotherapy treatments in Breast (BC) and Pancreatic Cancer (PC) have limited efficacy, highlighting the importance of finding new strategies to improve treatment outcomes and overcome resistance to current therapy. This mini review analyses whether manipulating the vasculature via proangiogenic factors in the Tumour Microenvironment (TME) of obese Pancreatic and Breast cancer tissue will improve obesity associated inflammation, metastatic behaviour, and outcomes of anti-VEGF therapy...


Capecitabine Induced Palmoplantar HyperpigMentation - A Rare Adverse Effect

Sindhusha Veeraballi*; Amr Ramahi; Gunwant K Guron

    A 77-year-old female with left breast cancer diagnosed in 2017 s/p adjuvant chemo with TC (Taxotere and Cyclophosphamide) x 4 cycles, s/p radiation and was on hormonal therapy with tamoxifen for 2 years developed right breast mass and serous discharge from nipple in August 2020. She was diagnosed with right breast triple negative invasive ductal carcinoma. She received neoadjuvant chemotherapy with Adriamycin, Cyclophosphamide (AC) followed by paclitaxel and radiation...


Nutraceutical Oncology: A Review

Okonkwo Cyril Onyedikachi*; Eze Emmanuel A

    Cancer is a public health concern, and a major source of global morbidity and mortality. The conventional cancer therapies such as systemic therapy, surgery and radiotherapy have brought concomitant adverse effects such as multidrug resistance, cytotoxicity to adjacent normal cells and high financial burden in accessing them. Hence, there is the need for a better and cost-effective approach in cancer management...


"Histological Classification of Primary Lung Cancer: A Study in Enam Medical College & Hospital, Savar, Dhaka, Bangladesh"

HN Ashikur Rahaman*; Shravana Kumar Chinnikatti; Md Ashiqur Rahman; Md Minuddin Hossain

    Background: Lung cancer has been the most common cancer in the world for several decades, and by 2008, there were an estimated 1.61 million new cases, representing 12.7% of all new cancers. Lung cancer is considered as the leading cause of cancer death among both men and women. It is also one of the most common cancers in the world. The majority of lung cancers are related to smoking.
Objective: This study was intended to identify the histopathological types of lung cancer in relation to gender, age, and smoking habit in a sample of Bangladeshi patients.


Lymphomatoid Drug Eruption Secondary to Tuberculin Skin Reaction

Amal Chakroun*; Makram Koubaa; Fatma Hammami; Khaoula Rekik; Chakib Marrakchi; Fatma Smaoui; Mounir Ben Jemaa

    Introduction: The Tuberculin Skin Test (TST) is a skin test for tuberculosis. Although it is deemed benign, incidents can occur. We report a rare case of Lymphomatoid Drug Eruption (LDE) secondary to TST.
Case Presentation: A 31-year-old man with no particular pathological history had a TST injection in the right forearm as part of a hiring assessment. After 72 hours, the TST was positive (10/15 mm). Few days later, the diameter of the induration gradually increased with release of serosities...


Simultaneous Malignancy of Primary Splenic Angiosarcoma and Low Grade B-Cell Lymphoma

Ayumi Numata*; Rika Sakai; Hideki Arakaki; Yasufumi Ishiyama; Megumi Koyama; Hirotaka Takasaki; Masaki Suzuki; Tomoyuki Yokose; Takanobu Yamada; Kazuma Yasui; Tetsuo Yoshida; Kazuho Miyashita; Shin Fujisawa; Naoya Nakamura; Hideaki Nakajima

    We present the first case of simultaneous Primary Splenic Angiosarcoma (PSA), an extremely rare and highly malignant vascular neoplasm, found under detailed examination for low-grade B-cell lymphoma. A 72-year-old male presented at our hospital with multiple lymphadenopathy of up to 15 mm, left pleural effusion, and marked splenomegaly. White blood cell count was within normal limit, but monoclonal B-cell population (1800/μL) was detected. Flow cytometry revealed CD3-, CD5+, CD10−, CD19+, CD20+, CD22+, CD23−, FMC7+, SmIgλ−, and SmIgκ−...


Tafro Syndrome from a Single Center Outside Japan: 3 Cases and Review of the Literature

Tolga Kuzu; Semra Paydas*; Behice Kurtaran; Saime Paydas; Emine Kilic Bagir; Melek Ergin M

    TAFRO is an interesting syndrome haracterized by thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. Originally it has been reported by Takai et al in Japan patients and later this entity has been reported out of Japan. Here we reported 3 cases with TAFRO from Turkey and reviewed availabla data, diagnostic difficulties and also treatment modalities.


Sickle Cell Trait and Blood Groups (ABO and Rh) in Angolans Submitted to Hemoglobin Electrophoresis

Euclides Nenga Manuel Sacomboio*; Dedaldina Tavares Sebastiao; Filomena de Jesus Correia Filho Sacomboio

    The sickle cell anemia is a chronic disease, incurable, although treatable, the number of cases of sickle cell anemia in Angola is considered worrying, in Luanda and it is estimated that around 16,000 babies are born with anemia in Angola each year sickle cell disease. Estimate the sickle cell trait and its relationship with blood groups (ABO and Rh) among Angolan users who perform hemoglobin electrophoresis in the Mediag laboratory.


Clinical phenotype and prognosis of JAK2 and CALR mutation in Asian patients with Essential Thrombocythemia

Chin-Hin Ng; Zhaojin Chen*; Christopher Ng; Elaine Seah; Eugene Fan; Grace Kam; Evelyn Siew-Chuan Koay; Yen-Lin Chee; Wee-Joo Chng

    Objectives: Calreticulin mutated Essential Thrombocythemia (ET) has a distinct clinical phenotype when compared to JAK2 V617F mutated ET. Here we determined the prevalence and compared the clinical phenotypes and outcomes of JAK2 mutated, CALR mutated, and both JAK2 and CALR unmutated (double-negative) genotypes in 331 Asian ET patients.


Minimal residual disease assessment in multiple myeloma

Camilo SG Humphrey; Alberto Rocci*

    The introduction of novel compounds such as the second-generation proteasome inhibitors, new immunomodulatory agents and monoclonal antibodies (mAb) determined a consistent improvement in the depth of response in patients with Multiple Myeloma (MM) [1]. A three drugs combination as induction followed by Autologous Stem Cells Transplant (ASCT) determines Complete Remission (CR) in more than 50% of the patients and this may increase if consolidation and maintenance are included following ASCT [2].


Comparison of time frame critical for feed supplement on haematological indices of azawak cattle in semi–arid zone of Niger Republic

Alassan NA*; Muhammad IR; Maigandi; Issa S

    The haematological parameters of apparently healthy of Azawak cattle consisting of 36 animals (18 bulls and 18 heifers) at CERRA, Maradi in semi–arid zone in Niger Republic were studied. Data were analyzed for the effect of the time frame critical for utilization of feed supplement per sex. According a time frame critical for utilization of feed supplementation (bull and heifer) higher and lower values of Packed Cell Volume (PCV) was obtained from S2DM (26.32±3.78%) and NS (23.44±4.26%), there was no significantly lower (P>0.05) for Azawak cattle breed in semi–arid zone. Haemoglobin (Hb) values was higher from S2DM (10.66±0.57 g/dl) and lower SMED (9.92±1.28 g/dl).


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