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Lung Cancer |

PAH or Non-PAH PH? FREE TO VIEW

Hala El Chami, MD; Ioana Preston, MD
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Tufts University School of Medicine, Boston, MA


Chest. 2015;148(4_MeetingAbstracts):600A. doi:10.1378/chest.2265559
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Abstract

SESSION TITLE: Malignant Disease Global Case Reports

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: We report a case of pulmonary hypertension in an adult female with T cell Large Granular Lymphocytic Leukemia (T-LGL Leukemia).

CASE PRESENTATION: 76 year old female, with past medical history significant for T-LGL since 2002, not requiring treatment, presented for progressively worsening dyspnea on exertion for two months (NYHA functional class III). She complained of increased shortness of breath and inability to perform activities of daily living. She had a remote 15 pack years smoking history. On physical exam, she had a loud pulmonic component of the S2 and elevated jugular venous distension. Her lactate dehydrogenase, platelets and white blood cell count had all been declining for the past 3 months. The cardiac stress test and pulmonary function test were normal. A transthoracic echo showed a dilated right ventricle with an estimated systolic pulmonary artery pressure (PAP) of 74mmHg. A ventilation perfusion scan showed a mottled appearance with small nonsegmental defects. CT angiogram of the chest showed bilateral apical infiltrates, enlarged pulmonary arteries and no pulmonary embolism. A right heart catheterization measured a mean PAP of 50 mmHg, a pulmonary capillary wedge pressure of 8 mmHg, a cardiac index of 1.92 L/min/m2, and a pulmonary vascular resistance of 13.6 WU. A diagnosis of pulmonary hypertension (PH) World Health Organization group 5 was given. She was started on low dose cyclophosphamide and prednisone for her T-LGL leukemia, and macitentan and sildenafil for her PH. Patient improved significantly both markers of leukemia and signs and symptoms of PH (NYHA functional class II).

DISCUSSION: PH associated with T-LGL leukemia is a rare entity which has been previously described only in 5 patients, two of which were in a French cohort of 229 patients with T-LGL leukemia. The simultaneous occurrence of clinical deterioration with hematologic and hemodynamic abnormalities in our patient suggests a relationship between the T-LGL leukemia and pulmonary vascular disease. It is known that patients with T-LGL leukemia produce high levels of proinflammatory cytokines and chemokines and have an altered immunity, features which are increasingly recognized in PH.

CONCLUSIONS: Like our case described here, all patients reported in the literature as having PH associated with T-LGL leukemia had severe PH on presentation, diagnosed by right heart catheterization. The PH resolved in all the cases once the T-LGL leukemia was treated. Although there are no data on specific treatments for PH associated with leukemia, given the severity of her PH, we elected to treat both the leukemia and the PH.

Reference #1: Lamy T, Bauer FA, Liu JH, Li YX, Pillemer E, Shahidi H, et al. Clinicopathological features of aggressive large granular lymphocyte leukaemia resemble Fas ligand transgenic mice. Br J Haematol. 2000;108(4):717-23.

Reference #2: Rossoff LJ, Genovese J, Coleman M, Dantzker DR. Primary pulmonary hypertension in a patient with CD8/T-cell large granulocyte leukemia: amelioration by cladribine therapy. Chest. 1997;112(2):551-3.

Reference #3: Bareau B, Rey J, Hamidou M, et al. Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases. Haematologica. 2010;95:1534-1541.

DISCLOSURE: The following authors have nothing to disclose: Hala El Chami, Ioana Preston

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