Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Mediastinal Lymphadenopathy in Homozygous Sickle Cell Disease: An Unusual Diagnosis FREE TO VIEW

Alok Bhatt, MBBS; Vinay Nidadavolu, MD; Alae Yaseen, MBChB; Aravind Ajakumar Menon, MBBS; Allan Ramirez, MD; Umair Gauhar, MD
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University of Louisville, Louisville, KY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1097A. doi:10.1016/j.chest.2016.08.1204
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that results in the “sickling” of erythrocytes, leading to hemolytic anemia and various other vascular complications. Mediastinal lymphadenopathy entails a variety of differential diagnoses. T-cell acute lymphoblastic leukemia/lymphoblastic leukemia (T-ALL/LBL) occurring in sickle cell disease is extremely rare.1

CASE PRESENTATION: A 34 year old African American male with known homozygous SCD who had recently been treated for pneumonia presented with cough and shortness of air. Plain radiography and computerized tomography (CT) of the chest showed right-sided consolidation with a moderate right pleural effusion, and significant mediastinal and hilar lymphadenopathy. Pleural fluid was exudative and cytology was positive for malignant T-cells. A bronchoscopy was performed and endobronchial ultrasounded (EBUS) guided fine-needle aspiration (FNA) of the demonstrated lymph nodes revealed abnormal T lymphocytes confirmed as T-LBL by flow cytometry. Subsequent bone marrow biopsy showed T-ALL. Appropriate chemotherapy was initiated.

DISCUSSION: Acute shortness of breath in patients with SCD can be due to acute chest syndrome (ACS), pneumonia, pulmonary embolism or pulmonary hypertension, among others. ACS can present with fever, cough, dyspnea, consolidation and pleural effusion2 - all of which were present in this patient. Mediastinal lymphadenopathy may occur secondary to infection including histoplasmosis in endemic areas and HIV; extramedullary hematopoiesis; malignancy; and granulomatous disease like tuberculosis and sarcoidosis (especially in African-Americans). Lymphadenopathy may also simply be reactive. The most common cause for a tumoral mass in SCD is extramedullary hematopoiesis.1 Pathological or microbiological confirmation is recommended in patients with mediastinal lymphadenopathy. There are some reports of patients with sickle cell disease or trait developing hematologic dyscrasias like myelodysplasia and acute myeloid leukemia, but no clear causal link has been defined either with the disease or hydroxyurea therapy.3 Only rare, usually single, case reports exist for co-existent hematologic neoplasias in patients with SCD.1 Mediastinal tumors in T-ALL are usually responsive to chemotherapy.

CONCLUSIONS: It is important to apply a uniform diagnostic approach to shortness of air and recurrent pneumonia even in the presence of a condition known to be associated with the symptoms. Mediastinal lymphadenopathy offers a host of differentials, and EBUS provides a relatively safe avenue for tissue diagnosis.

Reference #1: Paydas S.Sickle cell anemia and hematological neoplasias.Leuk Lymphoma, 2002;43:1431-4.

Reference #2: Lonergan GJ, et al.Sickle Cell Anemia.RadioGraphics, 2001;21:971-94.

Reference #3: Castro O, et al.Hydroxycarbamide treatment in sickle cell disease: estimates of possible leukaemia risk and of hospitalization survival benefit.Br J Haematol, 2014;167:687-91.

DISCLOSURE: The following authors have nothing to disclose: Alok Bhatt, Vinay Nidadavolu, Alae Yaseen, Aravind Ajakumar Menon, Allan Ramirez, Umair Gauhar

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