Potential efficacy of crizanlizumab in treating priapism in sickle cell disease: A case report.

This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.

Obstructive Sleep Apnea Does Not Exclude Polycythemia Vera: A Case Report.

Introduction: Polycythemia vera (PV) is one of the myeloproliferative neoplasms (MPN) diagnosed by World Health Organization (WHO) criteria 2016, which requires the presence of 3 major criteria: high hemoglobin/hematocrit, bone marrow findings, and Janus Kinase 2 (JAK2) mutation or two major and one minor criteria, including erythropoietin (EPO) level. However, in clinical practice, difficulties in diagnosis can arise as it may be masked by secondary causes for erythrocytosis such as smoking or obstructive sleep apnea (OSA). Case Presentation: Here, we report a 55-year-old gentleman, morbidly obese with OSA on home continuous positive airway pressure (CPAP) machine, who was incidentally found to have polycythemia. Further evaluation confirmed the diagnosis of PV. Conclusion: PV can be masked by the assumption of secondary polycythemia based on history. This underscores the importance of screening such cohort through JAK2 and EPO testing to avoid missing PV.

Myocardial Infarction as an Initial Presentation of Essential Thrombocythemia With Calreticulin (CALR) Mutation (None Type 1, None Type 2).

Essential thrombocythemia (ET) is one of the classical Philadelphia-negative myeloproliferative neoplasms with different mutations that can be associated with it, like Janus kinase 2 (JAK2), myeloproliferative leukemia protein (MPL), and Calreticulin (CALR) (types 1 and 2). However, there is a lack in the literature concerning other types of CALR mutations and their clinical significance and prognosis. Here we report a 42-year-old male with type 2 diabetes who presented with an inferior ST-elevation myocardial infarction and thrombocytosis. The diagnosis of ET with CALR (neither type 1 nor type 2) was confirmed, which suggests the pathognomonic feature of this mutation.

A Challenging Case of a Patient With Immune Thrombocytopenic Purpura on Eltrombopag Who Developed Atrial Fibrillation: An Anticoagulation Dilemma.

A 61-year-old female, who was a known case of immune thrombocytopenic purpura (ITP) on eltrombopag, was admitted for atrial fibrillation (AF). Labs showed a platelet count of 116 × 103/µL. AF reverted to sinus rhythm by cardioversion. Therapeutic enoxaparin was started for two days. She was discharged on dabigatran for four weeks. The choice of anticoagulation in these cases (ITP and AF) is not straightforward and needs further research.

Recurrence of acute chest syndrome post stopping Crizanlizumab, the dilemma of stopping vs continuation in patient with sickle cell disease: case report.

Sickle cell disease (SCD) is one of the most common hematological diseases, which results in variable complications. The treatment of SCD is evolving but limited options are available for now. Acute chest syndrome (ACS) is one of the serious complications observed in SCD and a challenging one in prevention. Crizanlizumab is a monoclonal antibody that binds to P-selectin and improves blood flow by preventing sickle cell adhesion to endothelium, resulting in improvement of vaso-oclusive crises (VOC). It is not well evaluated in terms of ACS prevention. Here we report a 23-year-old patient with SCD and recurrent ACS; she was started on Crizanlizumab and she had no more ACS, but once she was off Crizanlizumab she developed ACS again, later Crizanlizumab was re-started, and the patient has improved significantly.

Toe Gangrene as the First Presenting Symptom of Essential Thrombocythemia: A Case Report.

Essential thrombocythemia is a myeloproliferative neoplasm. Thrombosis and bleeding complications are common with myeloproliferative neoplasms, particularly essential thrombocythemia and polycythemia vera. Here, we report the case of a 52-year-old female who presented initially with painful toe swelling and discoloration. Initial imaging showed a small abscess. An incision and drainage, and debridement of toe dry gangrene were performed twice in two months with no improvement in her complaint and worsening discoloration, ending in a toe amputation. Two years later, the patient was referred to a hematology clinic for a high platelet count. On review of her medical records, the patient had the same numbers during the initial presentation. The patient's condition was diagnosed retrogradely by a hematologist as essential thrombocythemia. This case sheds light on myeloproliferative neoplasm as a differential diagnosis in patients with atypical thrombosis. Thinking in such a way could have diagnosed our patient two years earlier.

Radioactive Iodine-Induced Chronic Myeloid Leukemia in a Patient With Graves' Disease: A Case Report.

Therapy-related leukemia is an increasing concern in hematology. One of these substances that showed to increase the incidence of leukemia is radioactive iodine (RAI). We report here a case of radioactive iodine-induced chronic myeloid leukemia (CML) in a patient with Graves' disease, although most cases in the literature were for thyroid cancer. Also, our patient received a very low dose, which is unique compared to previous case reports in the literature.

Applications of Artificial Intelligence in Philadelphia-Negative Myeloproliferative Neoplasms.

Philadelphia-negative (Ph-) myeloproliferative neoplasms (MPNs) are a group of hematopoietic malignancies identified by clonal proliferation of blood cell lineages and encompasses polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The clinical and laboratory features of Philadelphia-negative MPNs are similar, making them difficult to diagnose, especially in the preliminary stages. Because treatment goals and progression risk differ amongst MPNs, accurate classification and prognostication are critical for optimal management. Artificial intelligence (AI) and machine learning (ML) algorithms provide a plethora of possible tools to clinicians in general, and particularly in the field of malignant hematology, to better improve diagnosis, prognosis, therapy planning, and fundamental knowledge. In this review, we summarize the literature discussing the application of AI and ML algorithms in patients with diagnosed or suspected Philadelphia-negative MPNs. A literature search was conducted on PubMed/MEDLINE, Embase, Scopus, and Web of Science databases and yielded 125 studies, out of which 17 studies were included after screening. The included studies demonstrated the potential for the practical use of ML and AI in the diagnosis, prognosis, and genomic landscaping of patients with Philadelphia-negative MPNs.

Rare presentation of primary varicella zoster as fatal fulminant hepatitis in adult on low-dose,short-term steroid: Case report.

BACKGROUND: Varicella zoster virus presents clinically as primary (chickenpox) or secondary (herpes zoster) infection. Cutaneous and extracutaneous dissemination may occur, usually in immunocompromised patients. VZV hepatitis that progresses to fulminant hepatic failure is very rare and fatal. To the best of our knowledge, 9 cases have been reported to date, of which 7 were in immunocompromised adults, and only one patient was on short duration steroid therapy. PRESENTATION OF CASE: We present a 26-year old man who was admitted initially with acute abdomen as query persistent biliary colic. Later, he showed clinical and laboratory findings of VZV hepatitis that progressed rapidly despite maximal medical ICU support and he expired on day 3 of admission. CONCLUSIONS: Acute VZV infection may present as fulminant hepatitis. The presentation may initially be challenging for the diagnosis and should be considered if the patient has been in contact with a sick case. Low dose corticosteroid could carry a risk for fatal VZV fulminant hepatitis and should be used very cautiously especially with VZV patients' contacts. Further causative relationships remain to be established.