Plasmablastic lymphoma is described as a subtype of non-Hodgkin's lymphoma under the category of diffuse large B-cell lymphoma. It is classified by WHO as HIV-associated lymphoma of the oral cavity. Several cases have been reported in non-HIV patients with extra-oral involvement. The characteristic immunohistochemical markers are generally positive for CD138, CD38 and MUM1/IRF4, and negative or weakly positive for pan B-cell markersCD20, CD79a, PAX-5 and BCL-6. We report a rare case of orbital plasmablastic lymphoma with oral and oropharyngeal involvement where the immunohistochemical markers were positive for CD138, CD43, CD45, CD79a and MUM1. A small subset of markers was weakly positive for CD20, CD30 and κ, and negative for CD10, BCL-6, CD4, CD56 and Epstein-Barr virus-encoded small RNA (EBER). Our case reinforces the fact that plasmablastic lymphoma is a different entity of non-Hodgkin's lymphoma that cannot simply be classified under diffuse large B-cell lymphoma. It demands modification of the WHO classification.
SUMMARYChylothorax is most common on the left side owing to the position of the thoracic duct. Malignancy-associated chylothorax is not uncommon. However, bilateral chylothorax is rare and malignancy should be a consideration in absence of trauma. We report a case of a patient with follicular lymphoma who presented with bilateral pleural effusion; pleural fluid analysis confirmed chylothorax. The patient did not have any significant peripheral or axial lymphadenopathy, which made the diagnosis difficult in absence of histopathology. Pleural fluid analysis was negative for malignant cells, however, the flow cytometry markers were suggestive of follicular lymphoma. Primary effusion lymphoma, which could have been another possibility, was ruled out by the flow cytometry markers. We conclude that pleural fluid flow cytometry markers play an important role where there is no significant lymphadenopathy and in absence of histopathological diagnosis. This demands further evaluation.
BACKGROUND
Pernicious anaemia is an autoimmune disease caused by intrinsic factor antibody; it leads to vitamin B12deficiency and is marked by ineffective erythropoiesis. Haematological features reveal macrocytosis, hyperchromasia and hypersegmented neutrophils. Schistocytes are typically seen in microangiopathy, such as in thrombotic thrombocytopaenic purpura (TTP)/haemolytic uraemic syndrome or disseminated intravascular haemolysis (DIC). We report a case of a patient with severe anaemia who presented to the emergency room. Peripheral smear revealed macrocytosis, hypersegmented neutrophils and marked schistocytosis. The patient also had high reticulocyte count with high serum lactate dehydrogenase, elevated D-dimer, low fibrinogen and low haptoglobin. Vitamin B12level came back low and the presence of intrinsic factor antibody confirmed pernicious anaemia. ADAMTS13 level was noted to be mildly reduced, which raised the suspicion of the association of acquired TTP with pernicious anaemia. Acquired TTP is another autoimmune disorder and its association with pernicious anaemia needs further evaluation.
The etiology was unclear but differential included drug, toxin, and infection or enhanced immune activity while he was on reduced dose of immunosuppression. The modest rise in platelet counts on adjusted (increased) dose of steroid and tacrolimus would support an immune hypothesis. The onset of severe anemia may point to bone marrow failure or secondary engraftment failure. Testing for donor specific antibodies (DSA) to HLA A2 (locus of mismatch) was 0%, DSA was 95% for DR10, DQ6, DR53, DQ5, DQ1, and weak DQ2. He was maintained on a low dose of prednisone (10 mg daily) and Tacrolimus (0.5 mg daily) for the first several days. Subsequent Bone marrow findings were remarkable for 20% marrow cellularity, preserved erythroid and megakaryocytic compartment, but marked
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