Introduction Avascular necrosis (AVN) has been associated with hematological malignancies; nonetheless, it is extremely uncommon in patients with chronic myeloid leukemia (CML). AVN has been described as a presenting manifestation in few CML patients. In other cases, it has been linked to CML treatment, specifically interferon-alpha (IFN-α) and tyrosine kinase inhibitors (TKI). This article reviews the available published literature to shed light on the existing evidence focusing on the pathogenesis, clinical characteristics, and outcomes of AVN in patients with CML. Results after an extensive search in the literature, we found 17 cases of AVN of the femoral head (AVNFH) and four cases of osteonecrosis of the jaw (ONJ) in CML patients. We did not find any reported case of AVN with CML in other sites apart from the femoral head and jaw. They were reported in 18 published articles between 1984 and 2021. There was an almost equal distribution between male and female patients with approximately a 1:1 ratio and a median age of 39 years (ranging from 7 to 71 years). A total of ten patients had AVNFH as the initial presentation of CML. Hip pain was the presenting symptom in all cases. The outcomes among these patients were variable. Almost all of them achieved symptomatic improvement after starting CML treatment, with some of them showing complete resolution of pain. However, in three cases, persistent gait problems were reported, three patients underwent hip replacement surgeries, and one patient developed another AVNFH after the initiation of Interferon-alpha. Five patients developed AVNFH after interferon therapy. Of note, all of them were on hydroxyurea therapy, in addition to interferon-alpha. Among patients who received TKIs for CML, we found only three cases of AVNFH associated with different TKI (Table 5). Notably, blood counts were within the normal range at the time of diagnosis with TKI-related AVNFH.ONJ was reported in four cases between 2018 and 2021(Table 6), and all of them were linked to TKI therapy. One patient had a recurrence of ONJ a year after healing (patient 1), and two patients required surgical intervention (patients 3 and 4). Discussion leukostasis and thrombocytosis that usually accompanied CML is the main predisposing factor for AVN. Leukemic cells and platelet aggregations in the microvascular circulation can lead to the microthrombi formation and subsequent interruption of blood supply to the areas with poor collateral circulation, especially femoral heads. We found that AVN in CML patients is exclusively affected the femoral head and jaw. Also, there was an almost equal distribution between males and females with a 1:1 ratio. The most common presenting symptom was unilateral hip pain, even in the cases found to have bilateral AVN on MRI. The patients with AVNFH as a presenting manifestation of CML were children and young adults. In addition, WBC counts were strikingly elevated in patients who initially presented with AVNFH (above 90,000). In contrast, platelet counts were variable which might support that leukostasis plays a significant role in the development of AVN. As evidenced by our data, there was no reported case of AVN with Pegylated IFN-α use, indicating that pegylated Interferon is safer than standard Interferon, especially in patients at high risk for AVN. Concerning the three cases that developed AVNFH after TKI therapy, we noted that all of them were in complete hematological response (CHR). Regarding OJN in CML, it occurred after prolonged use of TKI (more than two years in all cases). Seven patients with AVNFH underwent hip surgery (six patients required hip replacement), and only one patient (out of 17) had a recurrence of AVNFH at the same site. Given the lack of sufficient data, we could not conclude whether AVN in CML has an adverse prognostic effect. Nevertheless, we can conclude that the clinical outcomes of AVN in CML were not worse than AVN associated with other conditions. Conclusion AVN in CML is a rare condition that carries a high morbidity rate and long-term sequela. Therefore, it should be highly considered in the patient with CML who presents with either hip or jaw pain. Early detection and proper management of AVNFH in CML patients are essential to prevent longstanding disability. Based on our review, we can conclude that the prognosis of AVNFH in CML is considered good. However, further research is required to clarify the effect of AVN on the prognosis of CML. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.
Rationale: Favism is a well-known cause of acute hemolytic anemia. Rarely, methemoglobinemia can also happen because of fava bean ingestion in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Few cases with this co-occurrence have been reported in the literature.Patient concerns: We report a case of a 47-year-old patient who presented with jaundice that started 2 days after eating fava beans.Diagnoses: Laboratory investigations revealed anemia with evidence of hemolysis (high reticulocytes count, high indirect bilirubin, bite cells in peripheral smear). Blood gases showed high methemoglobin level. Reduced level of G6PD enzyme confirmed the diagnosis of G6PD deficiency.Intervention: The patient was kept on supplemental oxygen. He was counselled to avoid food and drugs that can cause acute hemolysis.Outcomes: Oxygen saturation improved gradually. The patient was discharged without any complications after 2 days.Lessons: Patients with G6PD deficiency can develop both acute hemolytic anemia and methemoglobinemia secondary to fava beans ingestion. These patients should not receive methylene blue to avoid worsening hemolysis.Abbreviations: G6PD = glucose-6-phosphate dehydrogenase, Hb = hemoglobin, MetHb = methemoglobin, NADPH = nicotinamide adenine dinucleotide phosphate, SatO 2 = oxygen saturation.
Cardiotoxicity is damage to the heart muscle, which affects its function. Chemotherapy is known to cause cardiotoxicity along with many other medications and etiologies. Many chemotherapeutic cocktails are known to be associated with cardiotoxicities, such as taxane and cisplatin. Patients might have arrhythmias, severe bradycardia, cardiomyopathy, and even cardiac arrest, so precautions are taken when such medications are started. This report presents a patient who developed severe symptomatic bradycardia after receiving idarubicin and cytarabine and was managed conservatively, along with a literature review of this entity.
Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm classically described as triphasic disease: chronic, accelerated, and blast crisis. There are many unmet needs and unanswered questions about CML. Intermittent fasting in patients with CML on tyrosine kinase inhibitors is among these unmet needs. Here we report the effect of intermittent fasting on response to nilotinib as upfront in a 49-year-old female Muslim who fasted during Ramadan and took her medication once instead of twice daily and remained in major molecular response.
Introduction Aortic thrombosis is an uncommon condition with serious embolic complications. COVID-19 is currently recognized to be associated with both venous and arterial thrombosis. However, only a limited number of COVID-19 cases associated with aortic thrombosis have been reported in the literature since the beginning of the pandemic. Case presentation A 66-year-old lady was admitted to our hospital with acute ischemic stroke. Floating aortic arch thrombus was detected incidentally on CT imaging. Interestingly, the patient reported a history of fever and cough and was found to have COVID-19 pneumonia based on nasopharyngeal polymerase chain reaction (PCR) and imaging. The patient received three months of anticoagulant therapy, and repeated imaging study did not reveal any aortic thrombus. Clinical discussion COVID-19 related aortic thrombosis has been reported chiefly in severe cases. The SARS-CoV-2 can directly infect the endothelium of the vessels, which might explain the occurrence of arterial thrombosis in milder COVID-19 cases with the absence of the hyperinflammatory state. The management guideline for aortic thrombosis is scarce and based only on case reports and series. Conclusion Aortic thrombosis is a devastating condition that can be easily missed without clinical suspicion. Our patient developed acute ischemic stroke, most likely embolic originating from the aortic thrombus. The clinician should consider this condition in any COVID-19 patient presenting with thromboembolic events, such as stroke or acute limb ischemia. Further study is required to explain the pathophysiology of arterial/venous thrombosis in mild-moderate COVID-19 cases.
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