We describe a 62-year-old patient with a 4-year history of myelodysplasia who later developedstriking features that included massive splenomegaly, rapidly evolving visual loss and a sensorimotor polyneuropathy. This led us to consider the diagnosisof haemophagocytic lymphohistiocytosis (HLH). Upon further investigation, we found that he fulfilled the necessarydiagnostic criteria for HLH, including the presence of haemophagocytosis of erythroid precursors on bone marrow smear.
Objectives: Infectious agents triggering haemophagocyticlymphohistiocytosis (HLH) primarily involve the herpes virus group. We report a case of HLH precipitated by Plasmodium falciparum.
Materials and methods: Clinical and laboratory findings in a patient presenting with fever were collected. After confirmation of acute malaria, anti-malarial treatment was administered.
Results: Despite initial favourable evolution, the patient developed fever again together with a worsening of the haematological parameters and increased ferritin levels. A bone marrow biopsy confirmed the diagnosis of HLH.
Conclusion: This case illustrates that HLH should be considered in the differential diagnosis of acute malaria in patients with persisting fever and pancytopenia.
Mesenteric cysts are a rare nosologic entity, the diagnosis of which is complex due to their nonspecific presentation. They may emerge from any part of the mesentery and grow to any size, thus conditioning a wide range of clinical manifestations that renders them easily mistaken for different gastrointestinal pathologies. Diagnosis encompasses a mixture of clinical suspicion, imaging techniques and sometimes surgery, and curative treatment is based on complete surgical resection of the cyst. We hereby present a case of a mesenteric cyst that developed on the anterior abdominal wall of a 59-year-old man awaiting allogeneic bone marrow transplantation after being diagnosed with chronic myeloid leukaemia. He was admitted to the emergency room with complaints of an increased abdominal perimeter and increased weight, not associated with alterations to his dietary or physical exercise habits. Suspecting ascites in the context of leukaemic progression, the patient was admitted to the medical ward; however, subsequent study identified a mesenteric cyst as the most probable diagnosis and the patient was proposed to undergo surgery. He underwent laparotomic cyst excision without complications and the histological evaluation of the surgical specimen confirmed the diagnosis.
Light-chain deposition disease is a rare paraproteinaemia characterized by deposition of monoclonal light-chain immunoglobulins with a non-amyloid structure, most frequently affecting the kidney. The authors present the case of a 58-year-old man admitted due to haemorrhagic shock caused by spontaneous splenic rupture. Investigation showed coagulopathy, homogeneous hepatomegaly with elevated cholestasis enzymes but normal bilirubinaemia, and a nephrotic syndrome with monoclonal kappa light chains. Liver and bone marrow biopsies revealed light-chain deposition disease. Splenic rupture as the presentation of a paraproteinaemia is rare. The diagnosis of light-chain deposition disease should be considered in case of a monoclonal plasma cell disorder with Congo-red negative deposits on histology.
Infection with cytomegalovirus (CMV) with resistance to ganciclovir (GCV) is a therapeutic challenge in kidney transplant patients, because standard treatment options are nephrotoxic. We report the case of a kidney transplant recipient with GCV-resistant CMV disease, in whom letermovir, a novel inhibitor of CMV packaging, was administered off-label and prevented a relapse of disease once the CMV load was decreased by cidofovir. Furthermore, we observed significant drug interactions between letermovir and tacrolimus.
Lenalidomide is an effective therapy against malignant plasma cells and a potent agent against proinflammatory and proangiogenic cytokines. The use of lenalidomide in POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein with plasma cells, skin changes) has been reported, but its benefit in long-term use is not well established. A 55-year-old man with POEMS and debilitating polyneuropathy was treated with lenalidomide and dexamethasone followed by maintenance lenalidomide. He remains in haematologic remission and in complete recovery of functional status 3.5 years after diagnosis. This case supports the long-term use of lenalidomide in patients with POEMS syndrome.