Only four percent of patients with non-Hodgkin’s lymphomas (NHL) present with lung involvement (12% with HL), but the disease should be included in the differential of lung lesions as its presentations are myriad. A rare case of anaplastic lymphomas kinase negative (ALK-) anaplastic large cell lymphoma (ALCL) of the skin involving the lung and mimicking Wegener’s granulomatosis (WG) is presented.
The patient is a 54 yo man with a 4-month history of ulcers on his nose bridge, left leg and arms. They started as round nodules. He had testicular cancer with left orchidectomy in 1987. Two years ago he received chemotherapy for NHL involving the neck and was in remission. He had weight loss and anorexia for 6 months. An HIV test was negative 2 years ago. He had an 80-pack year smoking history.He was thin, had dry, pale mucous membranes, no icterus, thrush or adenopathy. Temperature was 101.1F, pulse 120 regular, respiratory rate 16 and BP 94/78. He had a nasal bridge ulcer involving the cartilaginous septum. There were 1 to 2 cm, hyperpigmented, stage 2 ulcers on the extensor surface of the extremities. The remainder of the exam was normal.Labs are shown in table1. The diagnoses were sepsis, infected ulcers and dehydration. The differentials were syphilis, leprosy, HIV infection and WG. He received ceftriaxone and intravenous fluid. Skin and nasal biopsies were performed. He developed respiratory distress, was intubated and treated for septic shock. (Lab trends, graphs 1-5). The initial chest radiograph showed bilateral thick-walled nodules of varying sizes with cavitation, a right pleural effusion and a broken port-a-cath in the right descending pulmonary artery (figure 1). Later, there were superimposed alveolar infiltrates (figure 2, 3). Chest CT revealed the same findings (figure 4,5,6). Bronchoscopy was performed. His catheter was removed. He developed candida albicans urinary infection and sepsis. Bronchial washings and catheter were culture negative. Despite aggressive therapy, he died of septic shock on day 34.
The biopsies (figure7) revealed atypical, lymphoid infiltrates of large pleomorphic cells with abundant cytoplasm and prominent nucleoli with admixed neutrophils (below). The neoplastic cells stained positive with CD3 and CD 30 markers. The CD 20 and CD 15 stains were negative. CD 3 stains T-lymphocytes, while CD15 stains neutrophils and Reed-Sternberg cells (RS). CD 20 stains B-cells and CD 30+ cells are HL, particularly RS and ALCL, the latter comprising 2-8% of adult lymphomas. The ALK was negative. These findings were consistent with ALK - ALCL. There were no granulomas or vasculitis.Stein in 1982 discovered a cytokine receptor CD 30 on large lymphoid cells. It is absent in normal tissue and expressed on HL. In 1994 some cells were found to express the ALK-1 protein. There are 3 types, primary systemic (ALK+ and ALK-) and primary cutaneous, which is ALK-. The secondary ALCL arises from HL and peripheral T- cell lymphomas. They are usually ALK-. The absence of ALK is associated with a poor prognosis, unless it is the primary cutaneous form.
The case is of interest for several reasons. It represents an unusual presentation of an uncommon lymphoma. It has all the features of primary cutaneous (ALK negative) ALCL, which rarely disseminates to the lungs. Also, this patient may have had secondary ALCL arising from prior lymphoma and/or its treatment. This is the second reported case of cutaneous ALCL associated with Hepatitis C. Fourthly this is the first case report of ALCL mimicking WG, with skin nodules, nasal ulceration and thick walled cavitary lung nodules. However pathology was absent. Lastly, cavitation is rare in secondary lung lymphoma, which again makes the case an interesting pulmonary finding.
Reverly John, None.