Blastic Plasmacytoid Dendritic Cell Neoplasm without Cutaneous Manifestation: A Case Report.

BACKGROUND As an uncommon malignancy with the highest prevalence in the elderly population, blastic plasmacytoid dendritic cell neoplasm or BPDCN is a hematologic disorder with unknown pathogenesis and devastating outcomes. This neoplasm usually manifests in the skin but can also involve the bone marrow, and less frequently the central nervous system (CNS). However, it does not exclude other organs and can even be associated with other malignancies. CASE REPORT Here, we discuss an interesting case of BPDCN in an 85-year-old man who mainly presented with dizziness and weakness. Physical examination revealed splenomegaly, laboratory tests showed pancytopenia, and peripheral blood smear depicted metamyelocytes. Further workup including bone marrow biopsy revealed atypical cells and flow cytometry disclosed 84% blasts positive for cluster of differentiation (CD) 4, CD53, and CD156 suggestive of BPDCN. Moreover, cerebrospinal fluid (CSF) studies came back positive for tumor plasmacytoid dendritic cells. The patient underwent chemotherapy with CHOP, mini-CHOP regimens, and venetoclax, as well as treatment for CNS involvement. He achieved remission, but unfortunately had a recurrence of the disease. Later he was admitted due to pneumonia with concomitant recurrent pulmonary effusions complicated by multiorgan dysfunction and subsequently died. CONCLUSIONS The diagnosis of BPDCN can be very challenging, and high clinical suspicion and intuition are required to reach the diagnosis, especially when patients do not present with cutaneous involvement. Concerning treatment options, novel therapies such as tagraxofusp, a CD123-directed cytotoxin, are emerging in the hope of decreasing the rate of mortality for this aggressive malignancy.

Cholecystitis Masquerading as Cardiac Chest Pain: A Case Report.

BACKGROUND Cope's sign is the association of bradycardia with symptoms of acute cholecystitis, which can occur due to a vagal cardiobiliary reflex. The clinical and electrocardiographic changes of bradycardia or complete heart block can mimic the presentation of acute coronary syndrome. This report highlights the unique possibility that bradycardia in patients with abdominal pain and gallstones can be due to this reflex. CASE REPORT A 46-year-old obese man with hyperlipidemia and gallstones presented with chest pain suggestive of cardiac ischemia. The initial electrocardiography (EKG) was normal, although the patient subsequently developed bradycardia and a 2nd-degree atrioventricular (AV) block. The results of further cardiothoracic investigations (including echocardiography and pharmacologic stress testing) were normal. An ultrasound of the abdomen revealed acute cholecystitis. After he underwent a laparoscopic cholecystectomy, the chest pain resolved completely, and the EKG reverted to its normal sinus rhythm. CONCLUSIONS Acute cholecystitis rarely presents with cardiac chest pain and EKG changes due to triggering of the vagal cardiobiliary reflex. Given this atypical presentation, patients often undergo invasive cardiac procedures in search of a nonexistent cardiac etiology coupled with the possibility of a missed diagnosis of cholecystitis. When clinicians consider a diagnosis of acute coronary syndrome in patients with bradycardia, T-wave inversion, and ST-segment elevation (especially in the inferior leads), they should add the possibility of intra-abdominal pathologies (including cholecystitis) in the differential diagnosis.

The Clinical Challenges of Diagnosing Acutely Decompensating Amyloidosis.

Advanced amyloidosis and related multi-organ manifestations are devastating clinical scenarios. Because ambiguous presentation of amyloidosis may occur, early diagnosis and prevention of organ damage, such as cardiac injury, is essential and requires high clinical intuition. Our patient was a middle-aged female with a past medical history of heart failure with several decompensation episodes who presented with jaundice, itchiness, and weight loss. Further workup revealed pulmonary hypertension, restrictive heart disease, possible underlying obstructive liver disorder, and hyperkalemia. During admission, the patient established bradycardia and required a pacemaker temporarily, and later she manifested atrial fibrillation. Liver biopsy primarily was suggestive of hepatic congestion. Unfortunately, the patient died during workup due to cardiac arrest. Premortem laboratory results were suggestive of amyloidosis, which was confirmed later by re-examining the liver biopsy with Congo red. Diagnosis of amyloidosis requires early clinical suspicion and workup to prevent its progression to fatal organ involvement such as cardiac complications.

Neoplasia oncocítica adrenocortical de potencial maligno incierto: reporte de un caso / Adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report

Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.

Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.