Isolated Enteric Myeloid Sarcoma as a Rare Etiology of Small Bowel Obstruction in a Young Female Patient.

Myeloid sarcoma (MS) is an extra-medullary solid tumor consisting of myeloid blasts or immature myeloid cells. MS is usually associated with acute myeloid leukemia (AML) and other myeloproliferative neoplasms or myelodysplastic disorders. Isolated MS is a rare clinical entity, and the small bowel is a rare phenomenon for the occurrence of MS. A 30-year-old African American female patient with a past medical history of asthma presented with acute abdominal pain and vomiting for 3 days. Imaging revealed small bowel obstruction with a transition point at a suspicious mass in the distal ileum mimicking carcinoid tumors. She underwent an uneventful laparoscopic resection of this mass with primary bowel anastomosis. Histopathology of the resected mass revealed immature myeloid cells that stained positive for myeloperoxidase and CD34/CD117, in keeping with a small bowel MS. A bone marrow examination was negative for concurrent AML. Cytogenetic analysis revealed MYH11/CBFB fusion and an inversion 16 chromosomal aberration which are rarely associated with myeloid disorders. The patient was commenced on systemic chemotherapy to achieve remission and prevent progression to AML. The literature is reviewed, and all cases of small bowel MS are presented in this report. Non-leukemic small bowel MS is an exceptional presentation. We described a case of isolated enteric MS, which was associated with a rare MYH11/CBFB fusion and inversion 16 chromosomal aberration. The diagnosis of small bowel MS can be extremely challenging due to the rarity of the disease and non-specific nature of clinical and radiological features. A histopathological examination with immunohistochemistry staining is imperative to establish an accurate diagnosis. Isolated small bowel MS deserves special attention as it warrants systemic chemotherapy to prevent transformation into AML.

Successful Treatment of Persistent Coronavirus Disease 2019 Infection in a Patient With Hypogammaglobulinemia With REGN-COV2: A Case Report.

A 55-year-old man with hypogammaglobulinemia due to previous rituximab treatment developed persistent coronavirus disease 2019 pneumonia. Treatment with REGN-COV2 (casirivimab and imdevimab) resulted in the clearance of the infection. Targeted antiviral antibodies may be an important weapon in the management of immunocompromised patients infected with severe acute respiratory syndrome coronavirus 2 who fail to mount an immune response.

Posterior reversible encephalopathy syndrome in a patient with Richter's syndrome on combination DA-R-EPOCH chemotherapy regimen: a case report.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic entity characterized by headaches, altered mental status, seizures, visual loss, and a characteristic imaging pattern in brain magnetic resonance images. The exact etiology and pathogenesis of this condition are not yet fully elucidated. CASE PRESENTATION: A 72-year-old White man presented with 2 weeks of low-grade fever and chills, night sweats, fatigue, dysphagia, and new-onset rapidly increasing cervical lymphadenopathy. He had a history of chronic lymphocytic leukemia with transformation to diffuse large B-cell lymphoma for which he was started on dose-adjusted rituximab, etoposide, prednisone vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH). Shortly after treatment initiation, the patient developed severe airway obstruction due to cervical lymphadenopathy that required emergency intubation. A few days later, the cervical lymphadenopathy and the status of the airway improved, and sedation was consequently weaned off to plan for extubation. However, the patient did not recover consciousness and developed generalized refractory seizures. Brain magnetic resonance imaging revealed edema in the cortical gray and subcortical white matter of the bilateral occipital and inferior temporal lobes, consistent with PRES. CONCLUSIONS: Posterior reversible encephalopathy syndrome refers to a neurological disorder and imaging entity characterized by subcortical vasogenic edema in patients who develop acute neurological signs and symptoms of a usually reversible nature in different settings, including chemotherapy. Despite its name, PRES is not always fully reversible, and permanent sequelae can persist in some patients. Clinicians should be aware of the possible association between chemotherapy and PRES to ensure early recognition and timely treatment.

Usefulness of Mean Platelet Volume to Predict Significant Coronary Artery Disease in Patients With Non-ST-Elevation Acute Coronary Syndromes.

Acute coronary syndrome (ACS) is characterized by unstable plaque with thrombotic process involving central role of platelets. The diagnosis and prediction of significant coronary artery disease (CAD) in non-ST-elevation ACS (NSTE-ACS) can be challenging. The central role of platelets in acute atherothrombosis in NSTE-ACS spurred appreciable interest in the diagnostic and predictive role of platelet activity. Mean platelet volume (MPV) is one of the most promising laboratory markers in patients with CAD. This retrospective study was designed to investigate the diagnostic and predictive value of high MPV levels in patients with NSTE-ACS with significant CAD. A total of 213 patients (men 53%, mean age 61 ± 12.3 years) with NSTE-ACS were enrolled from 2011 to 2016 from 2 teaching hospitals. Patients' demographic, laboratory, and angiographic data were collected. Significant CAD was defined as ≥70% stenosis in at least 1 major coronary artery. Patients with high MPV (MPV ≥9 fl) had more significant CAD (55% vs 35%, p = 0.005), lower platelet count (204 ± 59 × 1,000/µl vs 246 ± 56 × 1,000/µl, p = 0.001), and higher HbA1c (6.9 vs 6.4, p = 0.02). Patients with significant CAD had higher MPV level (9.2 ± 1.07 vs 8.6 ± 1.03 fl, p = 0.001), higher MPV/platelet ratio (0.46 vs 0.40, p = 0.01), older age (64.5 ± 11 vs 59.2 ± 12 years, p = 0.02), and lower high-density lipoprotein level (42 ± 12 vs 47 ± 16, p = 0.01). Multivariate analysis showed that increased age, high MPV, high troponin, and low high-density lipoprotein levels were associated with significant CAD. Patients with high MPV along with high troponin level demonstrated a 4.8-fold increased risk for significant CAD compared to those with normal MPV and high troponin (odds ratio 4.8, 95% confidence interval 1.31 to 17.6, p = 0.001). In conclusion, considering high MPV in the context of elevated troponin level increases the predictive value of screening for significant CAD, and this result may help determine who is most likely to benefit from cardiac catheterization.

Hairy cell leukemia: towards a curative strategy.

Although not all patients who have HCL require therapy at diagnosis, most eventually need treatment. Historically, splenectomy and interferon-alpha resulted in hematologic responses; however, responses tend to be short. The introduction of purine analogs dramatically changed the prognosis for most patients who hae HCL. It is now considered standard of care to use a purine analog, such as 2-CdA or 2'-DCF, as first-line therapy. This approach results in a high CR rate and prolong DFS. Although both agents yield the same rates of CR and survival, 2-CdA seems easier to administer and my be associated with less toxicity. Despite the excellent results with purine analogs, most patients have MRD detected by sensitive techniques; 30% to 40% of patients eventually relapse and most require further therapy. A repeat course of 2-CdA (or 2'-DCF) will result in CR in approximately 70% of patients. For patients who have relapsed or refractory disease, monoclonal antibody-based therapies are emerging options. Rituximab and BL22 are highly active in this setting. Until BL22 becomes widely available, rituximab is a reasonable choice for salvage therapy; however, the dosing schedule needs to be denied further. The roles of rituximab and BL22 as initial therapy for patients who have previously untreated HCL have not been investigated. Fig. 1 is a suggested algorithm for the treatment of HCL. With the introduction of effective new agents, further studies will determine whether the now achievable prolonged survival will translate into cure.

2-CdA in the treatment of hairy cell leukemia: a review of long-term follow-up.

Hairy cell leukemia is a rare, indolent, chronic lymphoproliferative disorder characterized by the proliferation of a malignant B-cell clone with irregular cytoplasmic projections. Treatment with purine analogs such as 2-chlorodeoxyadenosine (2-CdA) is associated with excellent remission rates and long-term survival. Although the majority of patients achieve a complete remission (CR), most have minimal residual disease detected by sensitive methods. Despite the long term disease-free survival, approximately 36% of patients relapse and many require further therapy. Repeat administration of 2-CdA is very effective in achieving a second CR. Recently, new agents such rituximab and BL22 were shown to be effective in the treatment of relapsed and refractory disease.

A pilot study of denileukin diftitox (DD) in combination with high-dose interleukin-2 (IL-2) for patients with metastatic renal cell carcinoma (RCC).

High-dose (HD) IL-2 is approved to treat renal cell carcinoma (RCC) with modest response rates and significant toxicity. Enhancement of cytotoxic T-cell activity by IL-2 is 1 mechanism of action. IL-2 also stimulates regulatory T lymphocytes (Tregs), which are associated with poor prognosis. Favorable outcomes are associated with greater rebound absolute lymphocyte count (Fumagalli 2003). DD depletes IL-2 receptor (CD25 component) expressing cells. We hypothesized that sequential therapy could complement each other; DD would deplete Tregs so IL-2 could more effectively stimulate proliferation and activity of cytotoxic T lymphocytes. Patients (n=18) received standard HD IL-2 and 1 dose of DD daily for 3 days; periodic flow cytometry and complete blood counts were performed. Group A included 3 patients to assess safety only with DD 6 µg/kg between the IL-2 courses. Group B included 9 patients at 9 µg/kg DD before the IL-2 courses. Group C included 6 patients at 9 µg/kg DD between the IL-2 courses. Efficacy using the RECIST criteria was assessed after the treatment. Fifteen patients from a study of IL-2 without DD served as controls for toxicity comparison and 13 of these for flow cytometry comparisons. No unusual toxicity was noted. For group B/C patients receiving DD, the median decline in Tregs was 56.3% from pre-DD to post-DD (P=0.013). Peak absolute lymphocyte count change from baseline was +9980/µL for group B, +4470/µL for group C, and +4720/µL for the controls (P=0.005 B vs. C). The overall response rate was 5 of 15 (33%); 3 of 9 (33%) and 2 of 6 (33%) for groups B and C, respectively, including 2 patients with sarcomatoid RCC and 1 with earlier sunitinib therapy.