Inglês (pdf)
Artigo em XML
Referências do artigo
Tradução automática
Enviar este artigo por email
Citado por SciELO 
Similares em
SciELO 
Permalink
Case Presentation
We present the case of a 54-year-old male, a former smoker with a history of chronic cough, dyspnea, weight loss and asthenia. The physical exam revealed cervical lymphadenopathy. Laboratory tests revealed anemia, leukopenia and elevated IgM levels.
Contrast-enhanced CT of the neck, thorax, abdomen and pelvis demonstrated widespread lymphadenopathy (Figure 1), including a large retroperitoneal lymphomatous mass (Figure 2). In the pulmonary parenchyma, there were peribronchovascular ground-glass opacities and consolidation in the lower lobes (Figure 1). Additionally, a soft-tissue mass infiltrating the renal hila and perinephric fat was observed (Figure 3). The liver and spleen were enlarged.
The results of the bone marrow biopsy, integrated with the clinical and laboratory findings, confirmed the diagnosis of Waldenström macroglobulinemia (WM), a subtype of lymphoplasmacytic lymphoma (LPL).
Immunochemotherapy with bendamustine and rituximab was initiated. Follow-up CT showed near-complete resolution of the imaging findings.
Figure 1: CT images of the neck and thorax before treatment (A, C, E, G) and six months after immunochemotherapy initiation (B, D, F, H). Note the cervical (arrows on A), mediastinal (rectangle on C) and axillary (circles on E) lymphadenopathy and foci of consolidation (dashed arrows on G) with surrounding ground-glass opacities. The CT performed six months after treatment showed no cervical or axillary lymphadenopathy and resolution of the pulmonary findings.
Figure 2: CT images of the abdomen before treatment (A, C) and six months after immunochemotherapy initiation (B, D). A lymphomatous mass surrounding the inferior vena cava and aorta could be seen (arrows on A and C). Follow-up CT showed resolution of the mass, with residual mild fat stranding surrounding the great vessels.
Discussion
LPL is a B-cell neoplasm comprised of small lymphocytes, plasmacytoid lymphocytes and plasma cells and is usually associated with a serum monoclonal protein. WM is the most common subtype of LPL and occurs when the serum monoclonal protein is IgM and when there is bone marrow involvement. Elevated IgM levels can also be associated with other conditions, so a bone marrow biopsy is necessary for the diagnosis. Although not specific, most WM patients have a MYD88 gene mutation.
The incidence of WM is approximately 3.8 per million, making it a rare disease.1 The most common symptoms are constitutional and can be related to the infiltration of different organ systems by abnormal blood cells and blood hyperviscosity due to IgM hyperproduction.
Even though imaging is not required for the diagnosis of WM, it can have an important role in the diagnosis and patient management. Due to its multisystemic nature, WM can virtually affect any organ and lead to imaging findings in any system. Bone marrow involvement is the most frequent imaging manifestation, showing abnormal signal intensity on MRI of the spine.2 Extra-medullary manifestations are less common and may include lymphadenopathy (frequently in the abdominal region), splenomegaly and hepatomegaly.3 Rare extramedullary involvement may include renal or perinephric infiltration, nonspecific pulmonary manifestations such as consolidation or ground-glass opacities and, less frequently, involvement of other organ systems such as the gastrointestinal, musculoskeletal and central nervous systems.
All the previously described CT findings of our patient, some of them very uncommon, correspond to known manifestations of WM. Given the clinical context and concurrent disease findings, pulmonary lesions were attributed to WM without invasive biopsy. Although the role of imaging in WM is still uncertain, in our patient it contributed to the diagnosis, helped determine the extent of disease and was important in monitoring treatment response.
