Rheumatological picture of a patient having multifocal osteonecrosis associated with sickle cell anemia: a case study.

Am J Blood Res 2022;12(4):156-162 www.AJBlood.us /ISSN:2160-1992/AJBR0143160 Case Report Rheumatological picture of a patient having multifocal osteonecrosis associated with sickle cell anemia: a case study Albader Hamza Hussein1, Abdulhalem A Jan2, Lujain K Alharbi1, Khaled A Khalil1, Abdelrahman I Abdelrahman3,4, Salah Mohamed El Sayed5,6 Rheumatology Department, King Fahd Hospital, Al-Madinah Governorate of Health, Al-Madinah, Saudi Arabia; Cellular and Molecular Biology Division, Medical Laboratory, King Fahd Hospital, Al-Madinah Governorate of Health, Al-Madinah, Saudi Arabia; 3Department of Diagnostic Radiology, Jaber Al Ahmad Hospital, Kuwait; 4Department of Diagnostic Radiology, Zagazig Faculty of Medicine, Zagazig University, Egypt; 5Department of Clinical Biochemistry and Molecular Medicine, Taibah Faculty of Medicine, Taibah University, Al-Madinah Al-Munawwarah, Saudi Arabia; 6Department of Medical Biochemistry, Sohag Faculty of Medicine, Sohag University, Sohag, Egypt 1 2 Received March 28, 2022; Accepted June 16, 2022; Epub August 15, 2022; Published August 30, 2022 Abstract: Avascular necrosis (AVN) is a critical health condition associated with local death of the bone tissue resulting in multifocal osteonecrosis (MFON). After a prior patient’s consent, we present a case of sickle cell anemia associated with severe MFON that affected both long bones and short bones. She had a positive history of DVT. Initially, she presented with generalized severe bone pain with fever for seven days that got worse on the day of admission, a picture suggestive of sickle cell anemia-induced vaso-occlusive crisis. She was treated with adequate hydration, morphine, enoxaparin (a low molecular weight heparin), paracetamol and ceftriaxone. She got improved on treatment. On 5th day after admission, she developed sudden severe local tenderness at the distal tibia above the medial malleoli in both legs and she was unable to put a weight on her feet and could not stand up or walk. Plain X-ray films were not diagnostic. Complete liver function tests and kidney function tests were normal. The patient had leukocytosis, high serum urate and high serum LDH (may reflect cellular damage in bone cells). MRI scans revealed an evidence of bilateral multiple avascular necrosis in both femoral heads, left shoulder, left knee, and pelvic bones were evident. The patient’s condition was evaluated and the diagnosis of MFON associated with sickle cell crisis was established. This patient responded well to same treatments and her condition got improved. In conclusion, MFON should be considered after vaso-occlusive crisis of sickle cell anemia. Plain X-ray is non-conclusive in diagnosing bony lesions induced by AVN while MRI is diagnostic. Keywords: MFON, sickle cell anemia, leukocytosis, LDH, feet AVN, enoxaparin, uric acid, LDH Introduction Avascular necrosis is a critical health condition associated with local death of the bone tissue resulting in multifocal osteonecrosis (MFON) [1, 2]. MFON occurs more among some ethnic populations e.g. African Americans. The incidence is quite lower among Hispanic, non-Hispanic white and Asian/Pacific Islanders [3]. Osteonecrosis is a disabling disorder that frequently occurs in the younger population aged from 20 to 50 years [4]. MFON may occur in distinct anatomical sites e.g. the shoulder, knee, hip, ankle or where osteonecrosis results in disabling all these anatomical sites [5]. The use of corticosteroids has been reported as a major risk factor of MFON. Other risk factors include infection with human immunodeficiency virus, systemic lupus erythematosus, coagulation abnormalities, renal failure, multiple sclerosis, inflammatory bowel diseases, Sjogren’s syndrome, leukemia, lymphoma, and sickle cell disease [1, 2, 6]. Sickle cell disease is more common among African American children than among Hispanic American children. Sickle cell crisis is the acute painful crisis that consists of many phases: Phase I lasts about three days and is associated with a low-intensity aching pain. The patient may also report numbness and paresthesia. The aching increases rapidly in Phase II, which Rheumatological MFON associated with sickle cell anemia is due to local tissue infarct from vaso-occlusion. Phase III goes along with constant severe pain that may be associated with a fever due to post-infarct inflammatory responses. Phase III usually lasts three to five days. This is followed by the resolution of the vaso-occlusive crisis in phase IV over one to two days. Unfortunately, vaso-occlusive crisis may create a vicious cycle. Vaso-occlusive crisis often requires hospitalization due to several acute associated conditions e.g. aplastic crisis, splenic sequestration crisis, hyperhemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome. Other acute complications of sickle cell disease include avascular necrosis, osteomyelitis, stroke, pneumonia, meningitis, sepsis, priapism, and venous thromboembolism [7-9]. MFON diagnosis is challenging since most patients do not display the classic symptoms, making it difficult to identify the disease’s incidence for patients with other underlying diseases. Unfortunately, plain X-ray films are not so sensitive to diagnosing asymptomatic MOFN. The gold standard procedure for diagnosing MOFN is Magnetic Resonance Imaging (MRI) because of its sensitivity, accuracy, and specificity [10]. MRI limitations include high costs, time consumption and the need for optimizing the procedure in screening symptomatic and asymptomatic patients. Short-time inversion recovery (STIR) is a diagnostic procedure recommended to supplement MRI [11]. However, for sensitivity and accuracy, more research is required to develop more informed screening procedures. MFON has no typical symptoms. However, MFON presentation may depend on the affected joints sites e.g. the hip, knee, elbow and ankle in addition to affected bones as the calcaneus, and the tarsal navicular bones [12]. The most symptomatic sites are the knee, ankle, and hip. The symptoms are characterized by pain and lesions where the total number of lesions is higher in the hip, followed by the knee and shoulder at least among these three anatomical sites [1, 2, 6, 10, 11]. These osteonecrotic lesions appear consecutively among patients with high doses of corticosteroids. Based on other risk factors e.g. sickle cell disease and systemic lupus erythematosus, the total number of lesions follows the same sequence. The lesions are observed following a radiographic evaluation, showing the joints 157 affected by the disease. Seijas et al. note that femoral head’s necrosis is more prevalent among older persons [13]. Lamb et al. noted that the impact of MFON might be due to the weight impacted o the hip bone, increasing the risk of lesions or an injury [14]. This case study aims to shed light on the relevant predisposing factors, suitable diagnostic imaging tools and effective therapies of AVN. 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