Dyspnea and pulmonary nodules are common reasons for pulmonary consultation. A thorough history and physical examination with basic supplemental testing can often significantly narrow your differential diagnosis and suggest more unusual diagnoses or secondary processes.
A seventy-two year old male presents to pulmonary clinic with two to three years of insidious onset dyspnea on exertion. He first noticed on subway stairs, but now with one flight of stairs or any running. He has also had six months of decreased appetite, early satiety, and 10lb weight loss. He denies fevers, chills, night sweats, chest pain, cough, hemotptysis, lower extremity edema, paroxysmal nocturnal dyspnea, orthopnea, change in vision, easy bruising, bleeding or epistaxis. He has a >60 pack year history, but quit one year ago, occasional alcohol and marijuana usage, no intravenous drug usage or blood transfusions. He was born in New York City and has lived there for all but one year spent living throughout Europe in 1965—Canary Islands, Spain, Italy, France, and Switzerland –as well as Arizona for his service time in the Army and Marine Air Reserves. He is a heterosexual male, divorced multiple times without children. He had worked as a film director, painter, and in art studios with rubber cement and thinner exposure, as well as volunteering at a correctional facility until 2001. His PPD converted in 2004, but was never treated. On exam, he was thin and pale, with normal vital signs and no exertional desaturation. His labwork demonstrated a normocytic anemia and a white blood cell count of 10,700 with a differential with 6% atypical lymphocytes, but a peripheral smear with rouleaux formation. He also had an ESR of 105mm/hr, and an elevated total protein/albumin ratio. His imaging demonstrated a right middle lobe nodule, a right upper lobe ground glass opacity (GGO), and diffuse emphysematous changes and micronodularity. His workup revealed a monoclonal Igm kappa spike with 10,850mg/dL. His serum viscosity was 3.8 and beta-2 microglobulin level 3.4. His peripheral blood, bone marrow, BAL and transbronchial biopsies all demonstrated monoclonal IgM kappa positive B-cell population consistent with Waldenstrom’s macroglobulinemia.He began treatment with dexamethasone, rituximab, and cyclophosphamide. After the third cycle his repeat CT imaging demonstrated resolution of micronodularity, and improvement in GGO and nodule. Additionally his IgM levels trended down, and his exercise tolerance returned to baseline.
Waldenstrom’s macroglobulinemia was first described by Jan Waldenstrom in 1943, and demonstrates a lymphoplasmacytic B-cell lymphoma with intertrabecular bone marrow infiltration and an IgM monoclonal gammopathy. Pulmonary involvement has been reported to be present in 0–3% of all Waldenstrom’s cases, and its form is varied –masses, infiltrates, or effusions. Often pulmonary involvement is present at initial diagnosis, but it has also been reported as a later presentation. Symptoms often include dyspnea, though it has been hard to separate whether this is due to actual pulmonary involvement or anemia. Pathologic specimens have shown plasmacytoid lymphocyte infiltration, but notably there have also been reports of amyloid deposition. With the available data, pulmonary involvement does not seem to predict a worse prognosis.
Waldenstrom’s macroglobulinemia is a relatively rare diagnosis, and pulmonary involvement is reported even less frequently, but it is important to know the potential manifestations in order to expeditiously and properly diagnose our patients. Additionally we need more data in order to better determine the prognosis and outcomes of this diagnosis.
Eric Bonura, No Financial Disclosure Information; No Product/Research Disclosure Information