Acute thrombotic thrombocytopenic purpura in Louisiana: Seasonal distribution and evaluation of an ADAMTS13 order screening protocol.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by inactivation of ADAMTS13. It is characterized by thrombocytopenia and microangiopathic hemolytic anemia. Previous studies have produced conflicting data regarding seasonal association of TTP diagnoses. This study evaluated the seasonal distribution of acute TTP in southern Louisiana. Additionally, this study timeline overlapped with the initiation of a new screening protocol for ADAMTS13 testing. STUDY DESIGN AND METHODS: A retrospective analysis of patients receiving ADAMTS13 testing at Ochsner Medical Center from January 2010 to December 2017 was performed. TTP patient episodes were defined by ADAMTS13 activity <10%. Episodes were plotted according to season of presentation and a chi-square analysis was performed. Data of identified TTP patients was analyzed to evaluate for confounding variables. ADAMTS13 order volumes and results before and after screening protocol implementation were compared. RESULTS: Two hundred and fifty-two ADAMTS13 tests were ordered on 245 unique patients. Twenty-five patients had ADAMTS13 activity <10%. No significant differences in seasonal TTP case distribution were identified. Patients with confirmed TTP were younger and more likely to be Black or African American. A screening protocol did not decrease the number of annual ADAMTS13 test orders in our time frame, but was associated with a nonsignificant increase in the percentage of positive results. CONCLUSION: No significant seasonal distribution of TTP was identified in our population. This analysis provides previously unexamined regional information regarding this diagnosis. The number of tests ordered after the implementation of the screening protocol increased, corresponding with our facility's expanding reach.

Pathology Image of the Month: Abdominal Pain and Peripheral Eosinophilia.

A 69 year-old man presented to his primary care physician with abdominal discomfort. Medical history was notable for diabetes, chronic obstructive pulmonary disease with recent (one week prior) steroid use and hypertension. Surgical history was significant for a remote sigmoid hemicolectomy for diverticulitis with a synthetic mesh abdominal repair. He was admitted to the hospital for suspected gastroparesis. An upper GI series showed a distended stomach with delayed gastric motility. He underwent esophagogastroduodenoscopy and a duodenal biopsy was taken. He remained afebrile but had an elevated white blood cell count of 19.1 x 103/mcL (4.5 - 11.0 x 103/mcL) with 28.8 percent eosinophils on differential. Microscopic images of the duodenal biopsy are shown below.

Bilateral globus pallidus lesions.

Following a hypoxic-ischemic insult, the globus pallidus is selectively spared from ischemic injury in contrast to the caudate and putamen. The known causes for hemorrhagic and necrotic lesions selective for injuring the globus pallidus are varied but few. The most widely known etiology is in fatal cases of carbon monoxide poisoning. Additionally reported associations include fatalities involving 3,4-methylenedioxymethamphetamine; cocaine; opiates; and cyanide poisoning. These bilateral globus palladus necrotic lesions have been reported to occur in 5-10% of opiate addicts. In this article, we present a striking photo of selective symmetrical bilateral globus pallidus necrosis following cocaine and opiate abuse.

Progressive multifocal leukoencephalopathy: cavitary white matter lesions.

Progressive Multifocal Leukoencephalopathy (PML) is a fatal demyelinating disorder of the central nervous system caused by the opportunistic neurotropic Polyomavirus JCV. PML typically presents in immunocompromised individuals; is considered an AIDS defining condition; and is clinically characterized by cognitive defects, coordination problems, limb paresis, and speech disturbances. MRI can identify the characteristic bilaterally asymmetrical lesions that favor the subcortical frontal and parietal lobes as hypo-intensities on T1-weighted images and as hyper-intensities on T2-weighted images. Grossly, these areas appear discolored by a dusky, yellow-brown hue with the rare appearance of cavitation. No known therapeutic agent can specifically halt the progression of PML, and survival time after diagnosis averages less than two years. In this submission, we present a photo depicting striking lesions of severe PML of the cavitary type.