Management of Acute Myeloid Leukemia (AML) in Older Patients.

PURPOSE OF REVIEW: The acute myeloid leukemia (AML) treatment landscape has rapidly evolved over the past few years. These changes have several implications for the care of older adults (≥ 60 years), who have inferior clinical outcomes. We review decision-making in older adults, focusing on patient- and disease-related factors. We then summarize current treatment options, including multiple recently approved therapies, based on hypothetical clinical scenarios. RECENT FINDINGS: In lieu of using chronological age to determine fitness, we highlight the importance of standardized fitness assessments using geriatric assessments. Next, we review intensive and lower-intensity treatment options in the upfront setting. We focus on multiple newly approved medications, including venetoclax, midostaurin, CPX-351, gemtuzumab, glasdegib, enasidenib, and ivosidenib, and their specific indications. Lastly, we briefly discuss supportive care of older adults with AML. Outcomes of older adults with AML remain poor; fortunately, there are many new promising treatment options. Personalized treatment plans based on patient- and disease-specific factors are essential to the care of older adults with AML.

Testicular involvement in mantle cell lymphoma: An analysis of 16 patients.

Mantle cell lymphoma (MCL) with testicular involvement is a rare presentation and only a few cases have been described in the literature. We present a case of MCL with testicular involvement and the first analysis of all previously reported cases assessing trends in immunohistochemical features, prognostic indicators, and survival. Our data suggest that among all MCL, testicular MCL is more likely to present with aggressive features: blastoid/pleomorphic morphology, high Ki-67 proliferative index, and CNS involvement. Testicular MCL is also associated with shorter overall survival.

Referral to and receipt of allogeneic hematopoietic stem cell transplantation in older adults with acute myeloid leukemia.

INTRODUCTION: Recent data have shown improved outcomes in selected older adults with acute myeloid leukemia (AML) following allogeneic hematopoietic stem cell transplantation (HSCT). Nonetheless, practice patterns for referring and performing HSCT vary. We aimed to evaluate referral, utilization, and reasons for not referring/proceeding to HSCT in older adults with AML. MATERIALS AND METHODS: This is a single center retrospective analysis of patients aged ≥60 years diagnosed with AML evaluating rates of HSCT referral and utilization. Fisher's exact test was used to compare rates of referral and utilization across age groups and years of diagnosis. RESULTS: Median age of the 97 patients was 70 years (range 61-95); 30% (29/97) were referred for HSCT and of these, 69% (20/29) received HSCT. Common documented reasons (can be multiple) for not referring were performance status (n = 21), advanced age (n = 16), patient refusal (n = 15), refractory disease (n = 14), and prohibitive comorbidity (n = 6). Among patients who were referred but did not receive HSCT (n = 9/29), documented reasons for not proceeding with HSCT were refractory disease (n = 5), advanced age (n = 2), and prohibitive comorbidity (n = 2). HSCT referral and utilization rates significantly decreased with age (p < 0.01) but were generally stable over time from 2014 to 2017 (p = 0.40 for referral and p = 0.56 for utilization). DISCUSSION: Despite improvements in supportive care and HSCT techniques, HSCT referral and utilization rates remained low among older adults with AML but stable over time.

Acute Myocardial Infarction as Initial Manifestation of Acute Myeloid Leukemia: A Rare Manifestation of Leukostasis.

Leukostasis is a medical emergency caused by compromise of tissue perfusion secondary to hyperleukocytosis in acute myeloid leukemia (AML). Typically it affects lungs and brain, with cardiac involvement being exceedingly rare. We present a case of AML presenting as acute coronary syndrome secondary to leukostasis-induced myocardial ischemia. A 43-year-old morbidly obese gentleman presented with typical anginal chest pain. On examination, he was diaphoretic and in acute distress secondary to pain. EKG revealed ST elevation in lead I and aVL and PR depressions in precordial leads. Troponin peaked at 5.55 ng/mL. Echocardiogram showed normal left ventricle function with no wall motion abnormality. Blood work was notable for white blood cell (WBC) count of 185,200 cells/µL with 81% blasts. Coronary angiogram revealed no obstruction. Emergent leukapheresis and hydroxyurea were initiated. WBC count decreased to 48,200 cells/ µL and angina resolved after leukapheresis. With diagnosis of AML, he received 7+3 induction chemotherapy with cytarabine and idarubicin, followed by re-induction and consolidation chemotherapy. He subsequently underwent allogenic bone marrow transplantation and achieved complete remission. Hyperleukocytosis in AML can cause leukostasis, characterized by evidence of tissue ischemia. Coronary vasculature accounts for 6% of cases with leukostasis. This can manifest as myocardial infarction. Emergent and timely initiation of leukapheresis can potentially lead to a complete resolution of microvascular occlusion.

Metastatic Lung Adenocarcinoma With Occult Involvement of Gluteal Muscles as the Sole Site of Distant Metastases.

Lung cancer metastases to soft tissues are rarely reported in the literature. In this report, we discuss a case of a 59-year-old female who presented with worsening shortness of breath for over five months. A CT scan of the chest revealed right upper lobe mass and ipsilateral mediastinal adenopathy. An endo-bronchial ultrasound (EBUS)-guided biopsy of the involved lymph nodes revealed cellular features consistent with lung adenocarcinoma. MRI of the brain was negative for metastases; however, a positron emission testing (PET) scan showed fluorodeoxyglucose (FDG)-avid nodules in the soft tissues of the bilateral buttocks. Tissue biopsy of the buttock lesions confirmed metastases of lung origin. To the best of our knowledge, this is the first case report of metastatic lung adenocarcinoma with occult involvement of the gluteal muscles as the sole site of distant metastasis.

Metastatic adult Xp11.2 translocation renal cell carcinoma with TFE3 gene fusion in complete remission.

Xp11 translocation renal cell carcinoma (RCC) accounts for most pediatric cases of RCC but is uncommon in adults. It has an aggressive course in adults with poor response to chemoradiation. We describe a 64-year-old man with Xp11 translocation RCC who achieved complete remission following surgery and chemoimmunotherapy.

A Ligamentous Agony: Median Arcuate Ligament Syndrome as an Under-Recognized Cause of Abdominal Pain.

Median arcuate ligament (MAL) syndrome (MALS) is a rare clinical entity characterized by chronic abdominal pain resulting from compression of the celiac artery by the MAL. We present a case of MALS with imaging evidence of anterior compression of the celiac artery on expiration, which was relieved on inspiration. A 33-year-old woman presented with intermittent upper abdominal pain since three months. The pain was associated with nausea, abdominal bloating and diarrhea. Physical examination revealed a palpable abdominal aorta with no bruit. Abdominal sonogram, upper and lower gastrointestinal endoscopies, celiac disease screening, clostridium difficile toxin assays and hepatobiliary iminodiacetic acid scan were all normal. Computerized tomography angiogram (CTA) revealed subtle narrowing at the origin of the celiac artery without any atherosclerosis or calcification. Lateral aortic angiography showed anterior impression on the celiac artery at its origin on expiration. The constriction was relieved on inspiratory film - findings most consistent with MALS. MALS is rare, typically presenting with non-specific symptoms including nausea, vomiting, chronic post-prandial abdominal pain or unintentional weight loss. Angiography with respiratory maneuvers remains the diagnostic standard. However, non-invasive vascular imaging during both phases of respiration can be considered as an initial diagnostic test. The primary goal of therapy is celiac artery decompression with the additional objective of neuronolysis of the celiac ganglion. Our case highlights that MALS should be considered as a differential diagnosis in chronic, recurrent abdominal pain, particularly with an unrevealing initial evaluation. Evidence of celiac artery compression on vascular imaging, with characteristic respiratory variation, is highly suggestive of MALS.

Spontaneous tumor lysis syndrome in squamous cell carcinoma of the lung.

Tumor lysis syndrome (TLS) is an oncologic emergency, more commonly occurring in patients with hematologic malignancies receiving cancer-directed therapy due to massive cellular breakdown. Spontaneous TLS is rare and occurs in the absence of cancer-directed therapy. Herein, we present a case of spontaneous TLS associated with squamous cell carcinoma.