Hussein Albader Hamza, Jan Abdulhalem A, Alharbi Lujain K, Khalil Khaled A, Abdelrahman Abdelrahman I, El Sayed Salah Mohamed
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.
Am J Blood Res. 2022 Aug 15;12(4):156-162. eCollection 2022.
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 5 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.
缺血性坏死（AVN）是一种严重的健康状况，与骨组织局部死亡相关，可导致多灶性骨坏死（MFON）。在获得患者的事先同意后，我们报告一例与严重MFON相关的镰状细胞贫血病例，该病例累及长骨和短骨。她有深静脉血栓形成（DVT）的阳性病史。最初，她出现全身严重骨痛伴发热7天，入院当天病情加重，表现提示镰状细胞贫血引起的血管闭塞性危机。她接受了充分补液、吗啡、依诺肝素（一种低分子量肝素）、对乙酰氨基酚和头孢曲松治疗。治疗后病情有所改善。入院后第5天，她双下肢内踝上方胫骨远端突然出现严重局部压痛，无法负重，无法站立或行走。X线平片无诊断价值。肝功能和肾功能全套检查均正常。患者白细胞增多，血清尿酸和乳酸脱氢酶升高（可能反映骨细胞的细胞损伤）。磁共振成像（MRI）扫描显示双侧股骨头、左肩、左膝均有双侧多发性缺血性坏死迹象，骨盆骨也很明显。对患者病情进行评估后，确诊为与镰状细胞危象相关的MFON。该患者对相同治疗反应良好，病情有所改善。总之，镰状细胞贫血血管闭塞性危机后应考虑MFON。X线平片对诊断AVN引起的骨病变无决定性意义，而MRI具有诊断价值。
