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Hemic and Lymphatic Diseases - Top 30 Publications

Kimura disease of buccal region in a pediatric patient with nephrotic syndrome: A case report.

Kimura disease is a rare benign, chronic inflammatory disorder that typically presents with slowly enlarging, nontender, subcutaneous swellings in the head and neck region. The occurrence of Kimura disease in the oral cavity is extremely rare.

Cavernous hemangiomas of the temporalis muscle with prominent formation of phleboliths: Case report and review of the literature.

Hemangiomas are benign tumors characterized by an abnormal proliferation of blood vessels, most often occur in the skin and subcutaneous tissue, intramuscular hemangioma, a distinctive type of hemangioma within the skeletal muscle, account for <1% of all hemangiomas, temporalis muscle is a very uncommon site, cavernous hemangioma of the temporalis muscle with prominent formation of phleboliths is rare reported.

Secondary systemic lupus erythematosus after thymoma resection misdiagnosed as medically unexplained dyspnoea: A case report.

Secondary systemic lupus erythematosus (SLE) is an exceedingly rare complication of thymoma resection and is difficult to diagnose because of the insidious and nonspecific clinical manifestations. A case of SLE that occurs secondary to thymoma resection is described in this report.

Lymphedema secondary to idiopathic occlusion of the subclavian and innominate veins after renal transplantation: A case report.

Among the causes of swelling in the extremities of renal transplantation patients, secondary lymphedema caused by complete idiopathic obstruction of large veins is rare and may be difficult to diagnose and treat.

Male breast cancer and mantle cell lymphoma in a single patient: A case report and literature review.

Although still relatively rare, multiple primary malignant neoplasms (MPMNs) have been increasingly reported in recent years.

EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.

Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world.

The association between methionine synthase A2756G polymorphism and hematological cancer: A meta-analysis.

Numerous studies have focused on the association of methionine synthase (MS) A2756G polymorphism and acute hematological cancer risk. However, the results remain inconsistent. Therefore, a meta-analysis was performed to derive a more precise estimate of the association between them.

2017: a year in review.

Favism and Glucose-6-Phosphate Dehydrogenase Deficiency.

Ofatumumab for TTP in a Patient with Anaphylaxis Associated with Rituximab.

Reconstruction of the hip after resection of periacetabular oncological lesions: a systematic review.

Reconstruction of the acetabulum after resection of a periacetabular malignancy is technically challenging and many different techniques have been used with varying success. Our aim was to prepare a systematic review of the literature dealing with these techniques in order to clarify the management, the rate of complications and the outcomes.

Congenital respiratory tract disorders in 22q11.2 deletion syndrome.

Respiratory tract disorders have been reported in patients with 22q11.2 deletion syndrome, however infrequently. This study describes the respiratory tract disorders encountered in a cohort of 278 patients with 22q11.2 deletion syndrome.

Case 40-2017. A 32-Year-Old Woman with Headache, Abdominal Pain, Anemia, and Thrombocytopenia.

Obinutuzumab Treatment of Follicular Lymphoma.

Obinutuzumab Treatment of Follicular Lymphoma.

MBN 2016 Aesthetic Breast Meeting BIA-ALCL Consensus Conference Report.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon neoplasia occurring in women with either cosmetic or reconstructive breast implants. The actual knowledge about BIA-ALCL deriving from the literature presents several limits, and it remains difficult to make inferences about BIA-ALCL epidemiology, cause, and pathogenesis. This is the reason why the authors decided to organize an evidence-based consensus conference during the Maurizio Bruno Nava (MBN 2016) Aesthetic Breast Meeting held in Milan in December of 2016. Twenty key opinion leaders in the field of plastic surgery from all over the world have been invited to express and discuss their opinion about some key questions on BIA-ALCL, trying to reach a consensus about BIA-ALCL cause, pathogenesis, diagnosis, and treatment in light of the actual best evidence.

22 Cases of Breast Implant-Associated ALCL: Awareness and Outcome Tracking from the Italian Ministry of Health.

To date, 359 cases of anaplastic large cell lymphoma (ALCL) in women with breast implants (breast implant-associated ALCL [BIA-ALCL]) worldwide have been reported among more than 10 million patients who have received implants, but health care authorities suspect this is a possible underestimation, and the limited number of cases makes it difficult to clarify its cause. The General Directorate of Medical Devices and Pharmaceutical Services of the Italian Ministry of Health has examined and studied the Italian BIA-ALCL cases.

Paraneoplastic Auto-immune Hemolytic Anemia: An Unusual Sequela of Enteric Duplication Cyst.

Enteric duplication (ED) cysts are rare congenital anomalies of the alimentary canal that present in childhood. Although benign in most case, ED cysts have the potential to cause complications including a rare association with secondary carcinomas. Autoimmune hemolytic anemia presenting as a paraneoplastic syndrome secondary to solid tumors is an unusual phenomenon. Here we report a patient case with ED cyst described in association with intestinal adenocarcinoma and warm-auto immune hemolytic anemia, with resolution of the hemolysis upon its surgical resection.

Strong Prolyl Hydroxylase Domain 1 Expression Predicts Poor Outcome in Radiotherapy-treated Patients with Classical Hodgkin's Lymphoma.

Hypoxia-inducible factors (HIFs) and prolyl hydroxylase domain (PHD) proteins control cellular oxygen homeostasis and a wide range of other processes.

Distribution of Curcumin and THC in Peripheral Blood Mononuclear Cells Isolated from Healthy Individuals and Patients with Chronic Lymphocytic Leukemia.

Background/Aim: Curcumin is being widely investigated for its anticancer properties and studies in the literature suggest that curcumin distributes to a higher degree in tumor versus non-tumor cells. In the current study, we report on investigation of the distribution of curcumin and metabolism to THC in PBMC from healthy individuals and chronic lymphocytic leukemia (CLL) patients following exposure to Lipocurcâ„¢ (liposomal curcumin). Materials and Methods: The time and temperature-dependent distribution of liposomal curcumin and metabolism to tetrahydrocurcumin (THC) were measured in vitro in human peripheral blood mononuclear cells (PBMC) obtained from healthy individuals, PBMCHI(cryopreserved and freshly isolated PBMC) and CLL patients (cryopreserved PBMC) with lymphocyte counts ranging from 17-58×106 cells/ml (PBMCCLL,Grp 1) and >150×106 cells/ml (PBMCCLL,Grp 2). PBMC were incubated in plasma protein supplemented media with Lipocurcâ„¢ for 2-16 min at 37°C and 4°C and the cell and medium levels of curcumin determined by LC-MS/MS. Results: PBMC from CLL patients displayed a 2.2-2.6-fold higher distribution of curcumin compared to PBMCHICurcumin distribution into PBMCCLL, Grp 1/Grp 2 ranged from 384.75 - 574.50 ng/g w.w. of cell pellet and was greater compared to PBMCHIthat ranged from 122.27-220.59 ng/g w.w. of cell pellet following incubation for up to 15-16 min at 37°C. The distribution of curcumin into PBMCCLL,Grp 2 was time-dependent in comparison to PBMCHIwhich did not display a time-dependence and there was no temperature-dependence for curcumin distribution in either cell type. Curcumin was metabolized to THC in PBMC. The metabolism of curcumin to THC was not markedly different between PBMCHI(range=23.94-42.04 ng/g w.w. cell pellet) and PBMCCLL,Grp 1/Grp 2 (range=23.08-48.22 ng/g. w.w. cell pellet). However, a significantly greater time and temperature-dependence was noted for THC in PBMCCLL,Grp 2 compared to PBMCHIConclusion: Curcumin distribution into PBMC from CLL patients was higher compared to PBMC from healthy individuals, while metabolism to THC was similar. The potential for a greater distribution of curcumin into PBMC from CLL patients may be of therapeutic benefit.

Lymphedema of the Transplanted Kidney and Abdominal Wall with Ipsilateral Pleural Effusion Following Kidney Biopsy in a Patient Treated with Sirolimus: A Case Report and Review of the Literature.

BACKGROUND Sirolimus is a mammalian target of rapamycin (mTOR) inhibitor, which is used in immunosuppressive treatment regimens in organ transplant recipients. Although mTOR inhibitors are well tolerated, their adverse effects have been reported. Sirolimus treatment in transplant recipients has been reported to be associated with lymphedema of the skin and subcutaneous tissues, and with pleural effusion, but edema of internal organs and organomegaly have not been previously reported. A case is presented lymphedema of the transplanted kidney and abdominal wall with ipsilateral pleural effusion following kidney biopsy in a patient treated with sirolimus. CASE REPORT A 32-year-old woman with a history of end-stage renal disease of unknown etiology had undergone right renal transplantation from an unrelated living donor, eight years previously. She was referred to our hospital with dyspnea, localized abdominal pain, and swelling of the transplanted kidney. The symptoms appeared following a kidney biopsy and the replacement of cyclosporin with sirolimus four months previously. On examination, she had localized swelling of the abdominal wall overlying the transplanted kidney, and a right pleural effusion. Hydronephrosis and nephrotic syndrome were excluded as causes of kidney enlargement. Following the withdrawal of sirolimus therapy her symptoms resolved within three months. CONCLUSIONS A case is described of lymphedema of the transplanted kidney and abdominal wall with ipsilateral pleural effusion following kidney biopsy attributed to her change in anti-rejection therapy to sirolimus. This case report should raise awareness of this unusual complication of sirolimus anti-rejection therapy and its possible effects on the lymphatic system.

Single-cell dissection of monosomy 7 syndromes.

Etoposide for HLH: the limits of efficacy.

ALCL: is it now a curable disease?

Association of Marek's Disease induced immunosuppression with activation of a novel regulatory T cells in chickens.

Marek's Disease Virus (MDV) is an alphaherpesvirus that infects chickens, transforms CD4+ T cells and causes deadly lymphomas. In addition, MDV induces immunosuppression early during infection by inducing cell death of the infected lymphocytes, and potentially due to activation of regulatory T (Treg)-cells. Furthermore, immunosuppression also occurs during the transformation phase of the disease; however, it is still unknown how the disease can suppress immune response prior or after lymphoma formation. Here, we demonstrated that chicken TGF-beta+ Treg cells are found in different lymphoid tissues, with the highest levels found in the gut-associated lymphoid tissue (cecal tonsil: CT), fostering an immune-privileged microenvironment exerted by TGF-beta. Surprisingly, significantly higher frequencies of TGF-beta+ Treg cells are found in the spleens of MDV-susceptible chicken lines compared to the resistant line, suggesting an association between TGF-beta+ Treg cells and host susceptibility to lymphoma formation. Experimental infection with a virulent MDV elevated the levels of TGF-beta+ Treg cells in the lungs as early as 4 days post infection, and during the transformation phase of the disease in the spleens. In contrast to TGF-beta+ Treg cells, the levels of CD4+CD25+ T cells remained unchanged during the infection and transformation phase of the disease. Furthermore, our results demonstrate that the induction of TGF-beta+ Treg cells is associated with pathogenesis of the disease, as the vaccine strain of MDV did not induce TGF-beta+ Treg cells. Similar to human haematopoietic malignant cells, MDV-induced lymphoma cells expressed high levels of TGF-beta but very low levels of TGF-beta receptor I and II genes. The results confirm that COX-2/ PGE2 pathway is involved in immunosuppression induced by MDV-lymphoma cells. Taken together, our results revealed a novel TGF-beta+ Treg subset in chickens that is activated during MDV infection and tumour formation.

Nodal Involvement by CD30+ Cutaneous Lymphoproliferative Disorders and Its Challenging Differentiation From Classical Hodgkin Lymphoma.

Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma, representing almost 50% of primary cutaneous T-cell lymphomas, and primary cutaneous CD30+ T-cell lymphoproliferative disorders are the second most common group (30%). Transformed mycosis fungoides is usually CD30+ and can involve multiple nodal sites; other primary cutaneous CD30+ T-cell lymphoproliferative disorders can also involve draining regional nodes. Nodal involvement by CD30+ T-cell lymphoproliferative disorders can mimic classical Hodgkin lymphoma, which can aberrantly express T-cell antigens. The aim of this article is to briefly review salient clinical, histologic, immunophenotypic, and molecular features that can be used to distinguish lymph node involvement by CD30+ cutaneous T-cell lymphomas and lymphoproliferative disorders from classical Hodgkin lymphoma, a clinically important differential diagnosis that represents a challenging task for the pathologist.

Cardiovascular effects of Hodgkin's lymphoma: a review of literature.

Hodgkin's lymphoma (HL) constitutes 0.6% of all the cancers diagnosed worldwide and 10% of all lymphomas. Ten-year survival rate for HL is estimated to be 80% and cardiovascular death is the prevalent cause for a non-malignant death among these HL survivors. We are reviewing the cardiovascular morbidities associated with Hodgkin's lymphoma from the available literature. We performed a systematic review of all articles published from January 1, 1960 to October 1, 2016 on the MEDLINE database using PubMed search engine. Key factors in the diagnosis and management of these conditions are discussed in individual sections.

Role of splenectomy in the treatment of non-cirrhotic portal hypertension: about 3 cases.

Non-cirrhotic portal hypertension was first described by Guido BANTI in 1898 as a condition characterized by the association of portal hypertension with splenomegaly, anemia and healthy liver. The diagnosis was based on abdominal ultrasound, splenoportography and liver biopsy. Our study aimed to evaluate the role of splenectomy in non-cirrhotic portal hypertension. We conducted a retrospective study of 3 patients (2 women and 1 man) treated by our staff over the period January 2010 -September 2016. The diagnosis of idiopathic portal hypertension was based on the following criteria: portal hypertension, the presence of oesophageal varices associated with splenomegaly, the absence of cirrhosis or of other liver disorders responsible of portal hypertension. All patients underwent splenectomy. Outcome after splenectomy was marked by the standardization of clinical, radiological and biological signs of this disease associated with the absence of oesophageal varices recurrence. Splenectomy associated with ligation of oesophageal varices may be sufficient to treat this syndrome and especially its consequences without using splenorenal bypass.

Bacteremia due to Kingella denitrificans in a child followed-up for bone marrow failure syndrome.

Kingella denitrificans is a non-pathogenic micro-organism present in oropharyngeal flora. This germ has been recently recognized as responsible for opportunistic invasive infections mainly affecting immunosuppressed patients. We here report the case of a child aged 3 years and 7 months followed-up since the age of one year for bone marrow failure syndrome associated with pancytopenia of undetermined origin who had bacteremia due to Kingella denitrificans, a group of difficult to culture gram-negative bacteria rarely described in the literature. Clinicians and microbiologists should suspect the presence of this germ especially in immunosuppressed patients. The use of blood culture bottle contributes in a significant way to the detection of this germ.

Risk factors for retinal detachment in Togo.

Retinal detachment poses management problems in developing countries due to the shortage of technical equipment. Prevention passes through knowledge and elimination of risk factors. This study aimed to identify the risk factors for retinal detachment in people living in Togo.