Pernicious Anemia Presenting as a Mimicker of Thrombotic Thrombocytopenic Purpura.

A 52-year-old woman with no significant past medical history presented to the emergency room (ER) with nonspecific systemic symptoms, including fatigue, dyspnea on exertion, easy bruising, and palpitations. She was found to have significant pancytopenia. Hemolytic anemia, thrombocytopenia, and elevated PLASMIC score (6, High risk; PLASMIC = Platelet count; combined hemoLysis variable; absence of Active cancer; absence of Stem-cell or solid-organ transplant; MCV; INR; Creatinine) score at the time of presentation led to a concern for thrombotic thrombocytopenic purpura (TTP). Therapeutic plasma exchange (TPE) was deferred pending additional investigation. Workup revealed the true diagnosis of severe B12 deficiency, which would not have benefited from TPE and instead would have placed the patient at risk for harm, making the decision to defer treatment the correct and judicious approach. This is a case where anchoring on lab results may result in reaching the incorrect diagnosis. This case reminds clinicians of the importance of creating a broad differential and ensuring thorough history-taking is done for all patients.

The Speech "Bamana": Using the Syllable Repetition Task to Identify Underlying Phonological Deficits in Children With Speech and Language Impairments.

Purpose This study compared performance on the Syllable Repetition Task (SRT) by preschoolers with diverse speech and language abilities to identify underlying impairments in speech processes. Method Three groups of 13 children ages 4 and 5 years with (a) typically developing (TD) speech and language, (b) speech sound disorder (SSD), and (c) comorbid developmental language disorder and speech sound disorder (DLD + SSD) completed the SRT. We calculated competence, memory, encoding, and transcoding scores, as well as word-initial stress pattern and vowel accuracy. Results A 3 × 3 (Group × Syllable length) factorial multivariate analysis of covariance revealed group differences for all measures and syllable length differences for memory, transcoding, and competence. There were no interactions between group and syllable length. TD children obtained the highest scores on each measure, though children with DLD + SSD performed similarly to TD children on encoding when vocabulary was included as a covariate. Children with SSD only outperformed children with DLD + SSD on competence and transcoding, and these two groups performed similarly on memory. A separate exploratory analysis using a 3 × 3 multivariate analysis of covariance revealed that children with DLD + SSD were more likely than children in the other groups to produce weak word-initial stress and vowel errors during syllable repetition. Conclusion Children with SSD and DLD + SSD exhibit underlying phonological deficits on the SRT compared to TD children. Results support the claim that memory and encoding are deficits in SSD. In addition, transcoding deficits were identified among children with no known oromotor impairment. Therefore, more research is required to identify the relationship between SRT performance and explicit measures of phonological processing.

Comparison of Graft Acquisition and Early Direct Charges of Haploidentical Related Donor Transplantation versus Umbilical Cord Blood Transplantation.

Alternative donor allogeneic hematopoietic cell transplants (HCTs), such as double umbilical cord blood transplants (dUCBT) and haploidentical related donor transplants (haplo-HCT), have been shown to be safe and effective in adult patients who do not have an HLA-identical sibling or unrelated donor available. Most transplant centers have committed to 1 of the 2 alternative donor sources, even with a lack of published randomized data directly comparing outcomes and comparative data on the cost-effectiveness of dUCBT versus haplo-HCT. We conducted a retrospective study to evaluate and compare the early costs and charges of haplo-HCT and dUCBT in the first 100 days at 2 US transplant centers. Forty-nine recipients of haplo-HCT (at 1 center) and 37 with dUCBT (at another center) were included in the analysis. We compared graft acquisition, inpatient/outpatient, and total charges in the first 100 days. The results of the analysis showed a significantly lower cost of graft acquisition and lower total charges (for 100-day HCT survivors) in favor of haplo-HCT. Importantly, to control for the obvious shortcomings of comparing costs at 2 different transplant centers, adjustments were made based on the current (2018) local wage index and inflation rate. In the absence of further guidance from a prospective study, the cost analysis in this study suggests that haplo-HCT may result in early cost savings over dUCBT and may be preferred by transplant centers and for patients with more limited resources.

Primary CNS Burkitt Lymphoma: A Case Report of a 55-Year-Old Cerebral Palsy Patient.

With primary central nervous system lymphoma (PCNSL) being a rare disease, the subtype of Burkitt lymphoma (BL) presenting as a sole CNS lesion is an even more exceptional diagnosis. A case of coexistent primary CNS Burkitt lymphoma (PCNSBL) with cerebral palsy (CP) is presented. A 55-year-old Caucasian male presented with increasing bilateral lower extremity weakness above his baseline in addition to signs of increased intracranial pressure. Four abnormal enhancing masses were detected on MRI with biopsy results consistent with Burkitt lymphoma. Complete staging workup was completed with no evidence of extra-CNS disease noted on PET/CT, bone marrow biopsy, or cerebral spinal fluid analysis. The patient was treated with intravenous as well as intrathecal chemotherapy and found to be in a complete remission at six months. Recurrence in the CNS was observed four months later with treatment consisting of whole brain radiation as well as intrathecal chemotherapy. Thirty months after diagnosis, the patient remains disease-free. To our knowledge, this is the first case of PCNSBL in the setting of CP. A review of literature regarding treatment options in this controversial setting is provided.