Chylothorax as Rare Manifestation of Pleural Involvement in Waldenström Macroglobulinemia: Mechanisms and Management (original) (raw)
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Chylothorax as an Initial Manifestation of Waldenström macroglobulinemia
Cureus, 2020
Waldenström macroglobulinemia (WM) is an uncommon disease whose most common presenting features are anemia, hyperviscosity-related symptoms, B symptoms, bleeding, and neurological symptoms. Pulmonary compromise is rare, and there are a few cases reported of chylothorax as a manifestation of Waldenström macroglobulinemia. We present the case of a patient who presented with a refractory chylothorax as the initial manifestation of Waldenström macroglobulinemia.
Journal of community hospital internal medicine perspectives, 2018
Objectives: Lymphoplasmacytic lymphoma (LPL) is a mature B cell lymphoma that usually involves the bone marrow, spleen and lymph nodes. Extramedullary involvement, including the lung, is rarely reported. Case description: A 73-year-old female initially presented to our hospital complaining of productive cough of white-colour sputum for three weeks duration. She reported unintentional weight loss of ten pounds over the last five months. There was no history of haemoptysis, fever, night sweats, chills, recent infections or hospitalization. Chest imaging showed right lower lobe consolidation, small right pleural effusion. She was treated with oral antibiotic for pneumonia. After two months, a follow up chest imaging revealed persistent right lower lobe consolidation. Therefore, she was worked up for the possibility of malignancy. Bronchoscopy showed polypoid nodularities surrounded by black discoloured mucosa in the sub-segmental bronchi of the right lower lobe, and biopsy specimen revealed atypical B cell lymphocytic infiltrate. Polymerase chain reaction confirmed a clonal B-cell gene rearrangement supportive for a low-grade B-cell Lymphoma. Subsequently; serum immunofixation showed IgM of 1491 mg/dL (normal range 26-217 mg/dl) with normal levels of IgG and IgA. Urine contained free kappa light chains. Cytology with immunophenotyping of pleural fluid revealed lymphoplasmacytic lymphocytes. This combination of lab and bronchoscopy findings established the diagnosis of extramedullary Waldenström's macroglobulinemia. Conclusion: Waldenström's macroglobulinemia, a manifestation of LPL, is associated with an IgM monoclonal gammopathy in the blood. Extramedullary involvement including the lung is rarely seen in LPL. Physicians need to be aware of this rare presentation.
Journal of Medical Case Reports, 2020
Background Our case of a patient with untreated lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia with extramedullary pleural effusion is the first documented case of pleural fluid MYD88 L265P mutation status in a community hospital setting. Our patient was intolerant to 420 mg ibrutinib, but still achieved a lasting complete remission, as defined by National Comprehensive Cancer Network guidelines, with a dose reduction to 240 mg of ibrutinib. Case presentation A 72-year-old Caucasian (white) man diagnosed with monoclonal immunoglobin M kappa lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia monitored without treatment for 2 years, presented with dyspnea and a left pleural effusion. At presentation, computed tomography scans of his chest, abdomen, and pelvis showed layering left pleural effusion and para-aortic lymphadenopathy. Pleural fluid cytology demonstrated B-cell lymphoma of the lymphoplasmacytic subtype, with monoclonal kappa B-cell population on flow and ...
2019
Pleural effusion is a common finding in patients with advanced-stage lymphoma. Primary pleural lymphoma, instead, is a very rare entity that diagnosis is often challenging. This is a very interesting case of a 65-years-old immunocompetent woman affected by bilateral chylothorax as the only clinical manifestation of primary pleural small-B-lymphocytes non-Hodgkin lymphoma. The patients underwent to iterative thoracoscopic pleural biopsy since the mediastinal lymph-nodes biopsy and pleural effusion cytological examination were inconclusive. This case highlights the importance of a deep investigation of every pleural effusion, also with iterative pleural biopsy, because even an underestimated bilateral chylothorax can hide a rare neoplasm.
Primary Lung Involvement in Waldenström’s Macroglobulinaemia
Acta Haematologica, 2001
Pulmonary involvement in Waldenström’s macroglobulinaemia (WM) occurs in 3–5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.
An unusual case of Waldenstrom's macroglobulinemia presented with nasopharyngeal involvement
Ecancermedicalscience, 2013
We report a rare case of a 68-year-old male who presented with fever, weight loss, nasal blockage, and epistaxis. Examination revealed cervical and axillary lymphadenopathy with no evidence of organomegaly. On evaluation, bone marrow aspiration showed lymphoplasmacytic infiltration. The computed tomography of the neck showed nasopharyngeal mass and the biopsy of this mass and cervical lymph node showed lymphoplasmacytic lymphoma (LPL) with high serum IgM level. Hence, a diagnosis of Waldenstrom's macroglobulinemia (WM) was made. The patient received six cycles of chemotherapy with a combination of cyclophosphamide, vincristine, and prednisolone (COP regimen). Currently, the patient is under follow-up and in complete remission (CR), one year after completion of therapy. Nasopharyngeal involvement is extremely rare in WM, and hence we report this case.
Chylothorax in Patients With Chronic Lymphocytic Leukemia: A Case Series
Journal of Hematology
Chylothorax, which is defined as the presence of chyle in the pleural space, is often caused by malignancy. However, chylothorax as a result of underlying chronic lymphocytic leukemia (CLL) is exceedingly rare in the literature. Chyle contains fat soluble vitamins and lymphocytes, meaning that its collection into the pleural space may further exacerbate the immunosuppressed state of an individual with CLL. Here, we report three cases of patients with CLL who developed chylothorax, and their management. Chylothorax, although rare with CLL, should be considered in the differential diagnosis when patients with CLL present with pleural effusions, especially if recurrent. Discovery of a chylothorax may indicate the need for further treatment of CLL.
Revista de Chimie
The goal of pleural fluid assessment is to establish with certainty its etiology and major challenge is the etiologic diagnosis precocity. Pleural effusion as the first and the only event in the onset of non-Hodgkin lymphoma is atypically in the absence of other signs and symptoms. Follicular lymphoma (FL) also called indolent lymphoma the most frequent is a low-grade non-Hodgkin’s lymphoma (NHL). In patients with indolent lymphoma affecting the pleura and pericardium is atypical and appears only in aggressive forms, so the onset with pleural effusion is unusual. Serous effusions may occur from onset or during evolution of various subtypes of T cell originated lymphomas or high-grade B cell lymphomas. Primary pleural lymphomas (PPL) have been described at patients with human immunodeficiency virus infection; tuberculosis complicated with chronic pyothorax or after exposure to asbestos. Compared to other similar cases previously reported in the medical literature, the novelty of this...
Lymphoplasmacytic Lymphoma and Waldenström Macroglobulinemia
Archives of Pathology & Laboratory Medicine, 2013
Lymphoplasmacytic lymphoma (LPL) is a low-grade, B-cell neoplasm composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow, and it is associated with an immunoglobulin M (IgM) gammopathy. The definition of Waldenström macroglobulinemia (WM) and its relationship to LPL has been confusing in the past. In addition, the diagnosis of LPL itself can be challenging because LPL lacks disease-specific morphologic, immunophenotypic, and genetic features to differentiate it from other mature B-cell neoplasms. Accurate diagnosis of LPL/WM rests on recognition of the differential diagnostic features between LPL and other diagnostic possibilities and the use of the recently refined definition of WM and its relationship with LPL: The presence of an IgM monoclonal gammopathy of any level in the setting of bone marrow involvement by LPL. This review summarizes the clinical, laboratory, and histologic features of LPL/WM, with particular emphasis ...