The Curious Case of a Painful Leg Ulcer.

Sickle cell disease is a condition that can involve numerous organ systems secondary to vascular occlusion. Herein, we present a case of a 21-year-old male with sickle cell disease requiring long-term hydroxyurea therapy. Upon migrating to the United States from Yemen, the patient developed a rapidly progressive, exquisitely painful ulcer on his right lower extremity. Given his country of origin, a broad differential, including select infectious diseases, was essential. Moreover, establishing the unequivocally correct diagnosis was crucial to determine proper and safe therapy. Ultimately, a lesional biopsy demonstrated numerous sickled red blood cells occluding blood vessels, leading to the diagnosis of sickle cell disease-related leg ulceration.

Advancements in Sickle Cell Disease (SCD) Treatment: A Review of Novel Pharmacotherapies and Their Impact on Patient Outcomes.

Sickle cell disease (SCD) is a genetic hereditary blood disease that disrupts normal beta-globin production. Patients with SCD experience a broad range of symptoms ranging from anemia, pain crises, and jaundice to acute coronary syndrome and stroke. SCD has been treated with hydroxyurea since 1998. Three important pharmacotherapies have been approved by the Food and Drug Administration (FDA) in the past few years. L-Glutamine has shown efficacy in reducing vaso-occlusive pain crises and hospitalization. Crizanlizumab has also shown positive outcomes in patients with SCD. Voxelotor has been studied to be effective in improving hemolytic anemia and the quality of life in SCD patients. These drugs can be used alone or in conjunction with hydroxyurea. Trials have shown that these therapies have significant efficacy. The events of pain, hemolytic anemia, vaso-occlusive crises, and hospitalizations have been reduced by using these agents. In this editorial, we will discuss these advanced treatment options for patients with SCD.

Incidental Splenic Marginal Zone Lymphoma With Extreme Macrocytosis After Hydroxyurea Use: A Case Report.

Splenic marginal zone lymphoma (SMZL) is a low-grade mature B-cell lymphoma that typically presents in the form of splenomegaly and lymphocytosis. The diagnosis is traditionally made through splenic histology, the presence of circulating villous lymphocytes, or bone marrow biopsy. Its treatment can be in the form of chemotherapy, such as rituximab, or active surveillance. This case presentation discusses a 76-year-old female with a long history of hydroxyurea use for an unknown reason presenting with atypical symptoms requiring bone marrow biopsy to diagnose SMZL. This unique case demonstrates the importance of further research and studies into atypical SMZL presentations and hydroxyurea's potential in precipitating secondary malignancies.

A Case of JAK2V617F-Negative Myeloproliferative Neoplasm in a Young Female Presenting With Extreme Thrombocytosis.

Thrombocytosis is a commonly observed condition in clinical practice and typically results from various pathophysiological factors, such as iron deficiency, blood loss, infection, medications, rheumatologic conditions, malignancy, asplenia, post-splenectomy, or familial factors. However, extreme thrombocytosis, defined as a platelet count > 10,000 K/UL (equal or greater than a million), is a rare occurrence. In this report, we present a compelling case of severe thrombocytosis attributed to underlying chronic myelogenous leukemia (CML), further complicated by coexisting iron deficiency. It is essential to emphasize that not all instances of extreme thrombocytosis are indicative of essential thrombocythemia. Hence, maintaining a high level of suspicion for non-ET myeloproliferative neoplasms (MPNs) such as CML, as well as other underlying conditions like iron deficiency anemia, is crucial for accurate diagnosis and timely management.

FOETAL HAEMOGLOBIN (HbF) STATUS IN ADULT SICKLE CELL ANAEMIA PATIENTS IN IBADAN, NIGERIA.

AIM: Elevated HbF, among other biological and environmental factors, is responsible for decrease in mortality in sickle cell anaemia (SCA). This study determined the levels of HbF in adult SCA patients in steady state compared with HbAA controls. HbF was discussed in line with the clinical course of the disease so as to emphasize the relevance of hydroxyurea in the management of adult SCA patients. MATERIALS AND METHODS: The HbF levels of 66 confirmed SCA patients and 31 HbAA controls were estimated using Betke method and HbF percentage was calculated using formula: %HbF percentage = A413 filtrate x 100A413 standard x 20. RESULT: A statistical significant difference in the mean of the levels of HbF in patients (5.16±4.04) compared to controls (1.04±0.44) (p = 0.000) was observed. The mean levels of HbF for males (4.71±3.49) compared to that of females (4.99) were statistically similar (p =0.773). It was also observed that the mean HbF level appears to be declining as age advances. SCA patients were classified to three categories viz: HbF <2% (21.2% SCA patients); HbF of 2.1% -10%, (68.2% SCA patients); and HbF of 10.1% -16%, (10.6% SCA patients). CONCLUSION: Substantial proportion of our patients actually will require treatment with hydroxylurea to stimulate HbF production especially those with HbF percentage of <2 and some with HbF percentage of 2.1 - 10%. HPFH may be considered rare since only 10. 6% had HbF at the range of 10.1-16%. This study showed that in treating our SCA patients in Nigeria we need to adopt and encourage the use of HbF activating agents like hydroxyurea or any other safe agent that will be found to stimulate HbF production in SCD patients.