RESUMEN
Primary splenic lymphoma (PSL) is a rare disease and an improbable cause of splenomegaly or splenic nodules. On the contrary, splenic secondary involvement as part of an advanced lymphoproliferative disorder is more common. The authors present the case of a 49-year-old woman with a primary splenic diffuse large B-cell lymphoma (PS-DLBCL), in which the absence of other organs' involvement determined an ultrasound-guided biopsy of the spleen to achieve a definitive diagnosis. With this case the authors intend to emphasise the extensive differential diagnosis of splenomegaly, splenic nodules or infiltrates, the usefulness of splenic biopsy in establishing the diagnosis and recall a rare disease, with non-specific presenting symptoms, in which the diagnostic workup is challenging. LEARNING POINTS: The differential diagnosis of splenic nodules or infiltrates is vast and challenging, and it includes haematological diseases, systemic infectious diseases but also non-malignant infiltrative diseases.Although some lymphomas frequently present with splenomegaly, this is not the case of DLBCL, with the exception of PS-DLBCL.PS-DLBCL is a very rare pathology, accounting for 1% of all DLBCL and less than 1% of all NHL.
RESUMEN
A myeloid sarcoma is an extramedullary tumour arising from infiltration by leukemic cells at an anatomic site other than the bone marrow. Most commonly it precedes acute myeloid leukaemia but occasionally occurs simultaneously. It may also be associated with myeloproliferative neoplasms, myelodysplastic syndrome and the blast phase of chronic myeloid leukaemia. The most common sites for extramedullary tumours are bone, periosteum, soft tissue, lymph node and skin. Although this disease can affect a wide range of body sites, there are very few reports of peritoneal myeloid sarcoma or cavity effusion. The authors present the case of a 68-year-old man with myelodysplasia-related acute myeloid leukaemia and peritoneal myeloid sarcoma with myeloid ascites. The definitive diagnosis is challenging, requires a high level of suspicion, and relies on the exclusion of all alternative diagnoses and especially on complementary tests such as flow cytometry and immunohistochemistry analysis of ascitic fluid in order to detect the immature myeloid cells. LEARNING POINTS: Myeloid sarcomas are extramedullary leukemic tumours that occur before or simultaneously with acute myeloid leukaemia, other myeloproliferative neoplasms or myelodysplastic syndrome.Myeloid sarcomas are most often seen in bone, soft tissue, lymph node and skin, but can present in most locations.Peritoneal myeloid sarcoma and leukemic ascites, although very rare, must be searched for when a patient with acute leukaemia presents with newly diagnosed ascites, through ascitic fluid flow cytometry and immunophenotypic analysis.
RESUMEN
Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients the aetiology is still unknown, hence the designation of idiopathic MCD (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnoses excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD. We present the case of a 64-year-old man with iMCD (HIV- and HHV-8-negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratory remission was achieved and maintained at the 2-year follow-up. LEARNING POINTS: iMCD is a lymphoproliferative disease in immunocompetent patients with no known cause.To diagnose iMCD major and minor criteria must be fulfilled, and alternative diagnoses must be excluded.Nephrotic syndrome and motor polyneuropathy are rare in iMCD and all alternative diagnoses must be excluded before relating all of these.
