Castleman’s disease: an entity forgotten in ENT

<p class="abstract">Castleman’s disease (CD) usually presents as localized or systemic lymphadenopathy or as an extra nodal mass. The usual site of presentation are mediastinum, retroperitoneum, axilla and mesentery. Only 3 cases of CD have been reported in retro pharyngeal space. We report a case of 20 year old male patient with retropharyngeal mass. He presented with difficulty in swallowing, change in voice and respiratory distress. The mass was removed in-toto transorally after performing elective tracheostomy. The histopathological findings were consistent with hyaline vascular type of CD. He was decannulated after two day and postoperative period was uneventful. Postoperative CT imaging confirmed the complete excision of tumor and patient is on follow up, with no signs of recurrence. The presentation of tumour in the retropharyngeal space which is a rare site of occurrence add to the uniqueness of this case. Unicentric CD has an excellent prognosis and surgery is the management of choice. Its clinical features, histological subtypes, treatment modalities and prognosis are discussed.</p>

A severe case of thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly syndrome with myocardial and skeletal muscle calcification despite hypocalcemia: a case report

Background TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndrome is a recently recognized disease with a variety of presentations of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported. Case presentation A 24-year-old healthy young Asian man was admitted with intermittent epigastric pain and fever for 2 weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction. However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal muscle calcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization, with residual calcific lesions. Conclusion This is the first report of a patient with TAFRO syndrome and the complication of organ calcification. The etiology of calcification in this case is not clear. Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification. It is important to carefully handle the general management of TAFRO syndrome because of the possibility of various complications.

A Severe Case of Tafro Syndrome With myocardial and Skeletal Muscle calcification Despite Hypocalcemia: A Case Report

Background TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndromeis a recently recognized disease with a variety of presentation of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported.Case presentation A 24-year-old healthy young man was admitted with intermittent epigastric pain and fever for two weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction.However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal musclecalcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization with residual lesions of calcification.Conclusion This is the first TAFRO syndromereport with the complication of organ calcification. The etiology of calcification in this case is not clear.Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification.It is important to handle general management of TAFRO syndrome carefully because of various complications.

Excess leukocytosis (leukemoid reaction) associated with feline non-flea, non-food hypersensitivity dermatitis in a young cat

A one and a half-year-old, male, castrated, domestic short-haired cat was presented with a six‑month history of depression, anorexia, skin lesions, excessive itching and systemic lymphadenopathy. Complete blood count revealed severe leukocytosis (114,700 cells/μl), and peripheral blood films were characterized by marked lymphocytosis. Lymph nodes examinations and bone marrow aspirate were not suggestive of neoplastic changes. Histopathologic examination of skin lesions revealed allergic dermatitis. Based on the anamnesis and histopathologic features, non-flea, non-food hypersensitivity dermatitis (NFNFHD) was diagnosed. Treatment was initiated with prednisolone and cyclosporine. During the treatment, the cat fully recovered from the skin lesions. Leukocytosis was reduced to 18,940/μl six months after initiation of medication. To the authors’ knowledge, this is the first report describing a case of a leukemoid reaction secondary to feline NFNFHD.

Use of a Lymphatic Drug Delivery System and Sonoporation to Target Malignant Metastatic Breast Cancer Cells Proliferating in the Marginal Sinuses

Lymph node (LN) metastasis through the lymphatic network is a major route for cancer dissemination. Tumor cells reach the marginal sinuses of LNs via afferent lymphatic vessels (LVs) and form metastatic lesions that lead to distant metastasis. Thus, targeting of metastatic cells in the marginal sinuses could improve cancer treatment outcomes. Here, we investigated whether lymphatic administration of a drug combined with sonoporation could be used to treat a LN containing proliferating murine FM3A breast cancer cells, which are highly invasive, in its marginal sinus. First, we used contrast-enhanced high-frequency ultrasound and histopathology to analyze the structure of LVs in MXH10/Mo-lpr/lpr mice, which exhibit systemic lymphadenopathy. We found that contrast agent injected into the subiliac LN flowed into the marginal sinus of the proper axillary LN (PALN) and reached the cortex. Next, we examined the anti-tumor effects of our proposed technique. We found that a strong anti-tumor effect was achieved by lymphatic administration of doxorubicin and sonoporation. Furthermore, our proposed method prevented tumor cells in the marginal sinus from invading the parenchyma of the PALN and resulted in tumor necrosis. We conclude that lymphatic administration of a drug combined with sonoporation could exert a curative effect in LNs containing metastatic cells in their marginal sinuses.

Classic Hodgkin Lymphoproliferative Diseases Clonally Unrelated to B-Chronic Lymphocytic Leukemia Successfully Treated with Bendamustine Plus Rituximab

A 62-year-old male was diagnosed with B-CLL and treated with Fludarabine-containing regimen which maintained the disease in partial response. Nine years after diagnosis, a rapidly growing systemic lymphadenopathy was observed, and a biopsy specimen revealed the presence of typical Hodgkin/Reed&ndash;Sternberg (HRS) cells, surrounded by T-lymphocytes and CLL cells. Sequencing analysis of the germline CDR3 region of the IGH gene showed that the HRS cells were clonally unrelated to the preexisting CLL cells and the HRS cells were composed of five different clones, leading to the molecular diagnosis of de novo lymphocyte-rich classic Hodgkin LPDs with SLL. Because the initial treatment was neither effective for classic Hodgkin LPDs nor for SLL, Bendamustine, Rituximab (BR) was started and complete remission was achieved, which has continued for more than one year so far. BR may be a good therapeutic option for both entities without causing hematological toxicity.

Successful Treatment of Epstein-Barr Virus-Associated Lymphoproliferative Disorder with Rituximab in a Patient Undergoing Immunosuppressive Therapy for Aplastic Anemia

Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a currently emerging serious complication in immunosuppressed patients, especially in allogeneic transplant recipients. Several fatal cases of EBV-LPD have been reported in aplastic anemia (AA) patients receiving immunosuppressive therapy (IST) with antithymocyte globulin (ATG) plus cyclosporine A (CsA), but no appropriate prophylactic or therapeutic strategy has been established. Herein, we describe a 29-year-old man whose EBV-LPD was successfully treated with rituximab. He received IST with ATG plus CsA for hepatitis-associated AA. EBV-DNA in plasma, which was not detectable before IST, gradually increased after IST initiation. A high fever and systemic lymphadenopathy developed 31 days after IST initiation. An EBV-DNA titer of 5.7 × 105 copies/μl was detected, and a diagnosis of EBV-LPD was made. Although discontinuation of IST was not effective, a single dose of rituximab on day 33 resolved the clinical symptoms and completely eliminated EBV-DNA. Even after restarting CsA administration, no elevation of EBV-DNA was observed, and his complete blood cell count had fully recovered 1 year after IST. This case suggests that this treatment strategy for EBV-LPD with EBV-DNA monitoring and rituximab administration, which has been recommended in allogeneic transplant recipients, may also be useful in the context of AA patients receiving IST.