Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion.

BACKGROUND: Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi-organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub-optimal results, such as debilitating long-term neurological complications. Recently, few reports suggested that the addition of Therapeutic Plasma Exchange (TPE) might further improve the outcome. CASE DESCRIPTION: A 23-year-old woman with homozygote SCD was admitted with bony pains and vaso-occlusive crises. However, her course evolved to respiratory failure requiring mechanical ventilation, decreased level of consciousness, skin rash, severe anemia and thrombocytopenia and a picture consistent with thrombotic microangiopathy. MRI of the brain showed scattered multi-focal ischemic foci and cytotoxic edema. The patient received RCE on the third day after admission without improvement. On the seventh day, TPE was instituted (2 L/day of fresh frozen plasma for 5 days), following which she regained her consciousness and showed an improvement in her laboratory abnormalities. On follow up, she had gradual full neurological recovery and resolution of the MRI findings within a few months. CONCLUSION: FES remains a diagnostic and therapeutic challenge, with significant morbidity and mortality. Success in the management of this reported case with the addition of TPE to RCE supports the notion that TPE may be a potentially helpful modality that deserves further research.

Prevalence and Patterns of Renal Involvement Among Patients With Systemic Lupus Erythematous at a Tertiary Center.

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by widespread inflammation and damage to multiple organ systems. One of the most common and severe manifestations of SLE is lupus nephritis (LN). OBJECTIVES: To determine the prevalence of LN among subjects with SLE and to identify the demographic, clinical, and laboratory parameters of SLE in subjects diagnosed with LN. METHODS: This is a descriptive study conducted at a tertiary hospital. Medical records were reviewed from outpatients who visited between January 2015 and October 2019 and who has fulfilled the classification criteria for diagnosis of SLE and had LN. RESULTS: Among 365 patients with SLE, 36% had LN. The most prevalent World Health Organization class of LN was IV, which significantly correlated with both abnormal creatinine levels and nephrotic range proteinuria. Elevated serum creatinine correlated with the presence of hypertension and thrombocytopenia. Cutaneous manifestations were noted to be present in 100% of LN patients, followed by arthritis and/or arthralgia (82.9%), anemia (94.6%), and lymphopenia (87.6%). CONCLUSION: This study aids in the recognition of the demographic, clinical, laboratory features, and the histological patterns of LN patients in Saudi Arabia, that probably has a role in the development and disease progression. A significant correlation was found between abnormal kidney function and hypertension, thrombocytopenia and nephrotic range proteinuria. The presence of World Health Organization class IV LN correlated with both impaired kidney function and nephrotic range proteinuria.

Uterine Lipoleiomyoma: A Case Report and Review of Literature.

Uterine lipoleiomyoma is a rare benign neoplasm that falls under the umbrella of uterine leiomyoma. Upon histological examination, leiomyoma is primarily composed of smooth muscle cells of the uterus. Whereas, lipoleiomyoma reveals a characteristic pattern of mature adipocytes interspersed in uterine smooth muscles. This paper is discussing a case of a 60-year-old postmenopausal grand multiparous woman who presented with postmenopausal bleeding. A primary diagnosis of leiomyoma was made, however, histopathological diagnosis revealed its rare variant, lipoleiomyoma.