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Hemic and Lymphatic Diseases - Top 30 Publications

A rare case of important and recurrent abnormal uterine bleeding in a post partum woman caused by cavernous hemangioma: a case report and review of literature.

The cavernous hemangioma is a rare benign vascular tumor. About 50 cases of this disease were found in the literature over the last century and only 9 cases of cavernous hemangioma on the pregnant uterus were published it comes into cavernous or capillary form. The symptomatology is not unequivocal and when it occurs during pregnancy or postpartum, it causes life-threatening cataclysmic hemorrhage. Antenatal diagnosis is difficult and requires a multidisciplinary approach with pathologists, radiologists and gynecologists to avoid these complications or unnecessary hysterectomies. The diagnosis is histological. Hysterectomy is possible after failure of conservative treatment means. We report a rare case, a novel mixed cavernous hemangioma of the body associated with a capillary hemangioma of the cervix in a patient of 28 years 5th visors with recurrent genital bleeding in the postpartum period leading to a hysterectomy.

Unusual macrocystic lymphatic malformation in an adult patient.

Macrocystic lymphatic malformations (MLMs) constitute a circumscribed variation in deep lymphangiomas. They are characterized by a rare occurrence in adults, by their rapid expansion and by their frequent occurrence at the level of the cervicofacial region (75%) and axillary region (20%). They rarely occur in lower limbs. The diagnosis of MLMs is primarily based on clinical examination. Ultrasound has a valuable role in positive diagnosis and sometimes in differential diagnosis. MRI has a role in the diagnosis and the assessment of tumor extent, thus making a valuable contribution to surgery. Therapeutically, MLMs are mainly treated by sclerotherapy; surgery is rarely indicated. We report the case of 26-year old man with a one-year history of huge painless and rapidly evolving subcutaneous swelling in the right thigh. Clinical examination showed circumferential compressible non-beating infiltrated mass, surmounted by vegetative and ulcerated lesions. The remainder of the clinical examination showed the presence of inguinal ipsilateral lymphadenopathy, with mobility limitation of the right knee. Angio-MRI objectified diffuse heterogeneous gadolinium-enhancing cutaneous, subcutaneous thickening of the right thigh with perimuscolar aponeurotic involvement. Lymphoscintigraphy showed asymmetry of the accumultion of radiopharmaceutical kinetics that was reduced in the right lower limb in favor of an overload of the lymphatic system at rest. Given the clinico-radiological data, the diagnosis of MLM was retained. Given the seat of the lymphangioma and its extent, after consultation with the surgeons there was no indication for surgery. Immobilization with a removable bandage was indicated for our patient.

Occult hepatitis B reactivation in a patient with homozygous sickle cell disease: clinical case and literature review.

Occult Hepatitis B corresponds to the presence of hepatitis B virus-deoxyribonucleic acid (HBV-DNA) in serum and/or in liver of a patient despite HBsAg negativity. Clinically, it is usually asymptomatic. Its reactivation is rare and commonly occurs in immunosuppressed individuals. We report the case of a 21-year old patient from Senegal, with homozygous sickle cell disease, presenting with cholestatic jaundice. Laboratory tests showed reactivation of occult Hepatitis B. This study emphasizes the need to systematically investigate the presence of occult Hepatitis B in patients with sickle cell disease suffering from acute liver disease.

Meningococcal infections associated with febrile purpura among children hospitalized in a Moroccan Hospital: incidence and associated clinical factors.

Febrile purpura (FP) is suggestive of meningococcal disease, requiring almost always further investigations and a treatment based on broad spectrum antibiotics. This study aimed to determine the incidence of meningococcal infections as well as their associated clinical signs in children with febrile purpura hospitalized in the emergency department.

Pseudotumor cerebri revealing sarcoidosis.

Sarcoidosis is a multivisceral granulomatosis of unknown aetiology which may have various clinical and radiological manifestations. Cerebral sarcoidosis, although rare, can appears as a misleading pseudotumor. We report the case of a young Tunisian adult hospitalized for intracranial hypertension associated with pseudotumoral lesion on radiology, which was revealed to be systemic sarcoidosis.

Bean's syndrome in children: about two cases.

Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Radiological evaluation showed the presence of multiple lesions at the level of the jejunum and ileum consistent with small intestinal angiomatosis in the child aged 9 and a half years. It didn't show abdominal lesions in the child aged 5 years. The two patients were admitted to the operating block. Angiomas were surgically individualized. Some of them were actively bleeding. Enterotomy was performed. Postoperative sequelae was marked by the stop of the bleedings. This study aims to update the current understanding of this rare pathology as well as the benefit of surgical treatment in controlling the complications caused by this pathology and in reducing the frequency of transfusions.

A Gain-of-Function Mutation in EPO in Familial Erythrocytosis.

Familial erythrocytosis with elevated erythropoietin levels is frequently caused by mutations in genes that regulate oxygen-dependent transcription of the gene encoding erythropoietin ( EPO). We identified a mutation in EPO that cosegregated with disease with a logarithm of the odds (LOD) score of 3.3 in a family with autosomal dominant erythrocytosis. This mutation, a single-nucleotide deletion (c.32delG), introduces a frameshift in exon 2 that interrupts translation of the main EPO messenger RNA (mRNA) transcript but initiates excess production of erythropoietin from what is normally a noncoding EPO mRNA transcribed from an alternative promoter located in intron 1. (Funded by the Gebert Rüf Foundation and others.).

The clinical manifestation, survival outcome and predictive prognostic factors of 137 patients with primary gastrointestinal lymphoma (PGIL): Strobe compliant.

This retrospective study aimed to investigate clinical characteristics and prognostic factors in patients with primary gastrointestinal lymphoma (PGIL) of Chinese population.From January 2001 to December 2015, 137 patients diagnosed with PGIL were recruited. The clinical features, treatment, and follow-up information were analysed.The median patient age was 62.3 years. With 18.47 months follow-up, the 2-year progress-free survival and overall survival rate was 74.9% and 75.5%, respectively. The overall response rate was 33.6%. Age≥60 years, advanced Lugano staging (≥stage IIE), elevated lactate dehydrogenase (LDH) levels, ≥2 extra-nodal involved sites, National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI)≥4, Ki-67≥50% were associated with worse prognosis in univariate analysis (P < .05). By multivariate analyses, we determined that the involvement of extra-nodal involved sites was the only statistically significant poor prognostic factor in PGIL.Age, staging, LDH levels, NCCN-IPI, Ki-67 especially involvement of multiple extra-nodal sites were associated with poor overall survival of PGIL.

Effectiveness and safety of an optimized blood management program in total hip and knee arthroplasty: A large, single-center, retrospective study.

Little has been published on blood management in total hip and knee arthroplasty (THA and TKA, respectively) patients focusing on both hematopoiesis and hemostasis. Our aim was to explore the effectiveness and safety of an optimized blood management program in THA and TKA patients in a large, single-center, retrospective study.We retrospectively reviewed consecutive primary unilateral THA and TKA patients' data at our institution through the National Health Database. They were divided into 3 groups according to an optimized blood management program: group A-combined use of intravenous and topical tranexamic acid (TXA); group B-use of recombinant human erythropoietin (rHuEPO) and iron supplements in addition to treatments in group A; group C-use of additional multiple boluses of TXA in addition to treatments in group B. The primary outcomes were hemoglobin (Hb) drop and calculated total blood loss (TBL). Other outcome measurements such as transfusion rate, postoperative length of stay (PLOS), venous thromboembolism (VTE), and mortality were also compared.From 2014 to 2016, a total of 1907 unilateral THA (986 in group A, 745 in group B, and 176 in group C) and 1505 unilateral TKA (795 in group A, 556 in group B, and 154 in group C) procedures were conducted at our institution. The Hb drop, calculated TBL, and PLOS in group C were significantly lower than those in groups A and B for THA and TKA patients. The transfusion rate in group C was also significantly less than in groups A and B for THA patients, while it was similar in groups A and B for TKA patients. No patients in group C received a transfusion. A significant difference was not detected in the incidence of deep vein thrombosis. No episode of symptomatic pulmonary embolism or all-cause mortality occurred within 30 days postoperatively.The current retrospective study suggests that for patients receiving primary unilateral THA or TKA, multiple boluses of intravenous TXA combined with topical TXA, rHuEPO, and iron supplements can reduce the calculated TBL, Hb drop, transfusion rate, and PLOS without increasing the incidence of VTE or mortality.

Targeted next-generation sequencing identifies a novel nonsense mutation in SPTB for hereditary spherocytosis: A case report of a Korean family.

Hereditary spherocytosis (HS) is an inherited disorder characterized by the presence of spherical-shaped red blood cells (RBCs) on the peripheral blood (PB) smear. To date, a number of mutations in 5 genes have been identified and the mutations in SPTB gene account for about 20% patients.

Severe malnutrition evaluated by patient-generated subjective global assessment results in poor outcome among adult patients with acute leukemia: A retrospective cohort study.

To evaluate nutritional status in adult patients with acute leukemia (AL) using patient-generated subjective global assessment (PG-SGA) and to investigate the influence of nutritional status on prognosis.We observationally investigated 68 adult patients with newly diagnosed AL who received PG-SGA at the First Hospital of Jilin University between May 2013 and July 2015. Clinical features, chemotherapy regimens, biochemical indexes, body composition, complete remission (CR) rate, minimal residual disease (MRD), survival time, and side-effects of chemotherapy were compared between patients with and without severe malnutrition.Mean PG-SGA scores of the total patients were 6.1 ± 4.0, and 19 of 68 (27.9%) patients had severe malnutrition (PG-SGA score ≥9). Patients with acute myeloid leukemia (AML) had higher scores than those with acute lymphocytic leukemia (ALL; P = .011) and high-risk patients had higher scores regardless of whether they had AML or ALL (AML, P = .012; ALL, P = .043). Univariate analysis showed that severe malnutrition was correlated with age (P = .041), transferrin (P = .042), Karnofsky Performance Status score (P = .006), and C-reactive protein (CRP) (P = .018). Multivariate analysis demonstrated that severe malnutrition was associated with CRP (hazard ratio [HR] = 1.020, 95% confidence interval [CI]: 1.002-1.039, P = .026). No difference was found in CR rate (P = .831) between patients with and without malnutrition, but those who were severely malnourished had higher MRD (P = .048 in AML patients, P = .036 in ALL patients) and more gastrointestinal side-effects (P = .014). Severe malnutrition was also associated with inferior overall survival (HR = 0.243, 95% CI: 0.063-0.945, P = .041) but not with event-free survival (HR = 0.808, 95% CI: 0.338-1.934, P = .663).Severe malnutrition defined by PG-SGA in adult patients with de novo AL may result in poor outcome.

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD.

Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation.

Acute lymphoblastic leukemia arising after treatment of Ewing sarcoma was misdiagnosed as bone marrow metastasis of Ewing sarcoma: A case report.

Both acute lymphoblastic leukemia (ALL) and Ewing sarcoma (ES) are small round cell tumors, and it is difficult to differential diagnose them because of overlapping clinical, radiographic, histologic, and immunophenotypic features.

Isolated skeletal muscle recurrence of an originally nodal diffuse large B cell lymphoma: A case report and review of the literature.

Diffuse large B cell lymphoma (DLBCL) is a malignancy of the B cells with extranodal primary involvement being estimated at 30% to 40% of cases. Primary skeletal muscle presentation of DLBCL is extremely rare, with an estimated incidence of about 0.5% of extranodal lymphomas, presenting mostly in the lower extremities. The possible mechanisms of muscle involvement of DLBCL include primary extranodal disease, extension from adjacent organs (such as lymph nodes) or disseminated disease.

Synchronous Hodgkin lymphoma and gastric adenocarcinoma: A rare case report and literature review.

Hodgkin lymphoma (HL) is a lymphoproliferative disease arising in the lymphoid tissue, which is characterized by Reed-Sternberg cells. Adenocarcinoma is the most frequent pathological type of stomach cancer. Improved survival in HL patients leads to the development of secondary malignancies. However, synchronous occurrence of these 2 malignancies is extremely rare. Here, we present a 45-year-old male complaining of a lymph node mass in the neck, without any abdominal symptoms, diagnosed as HL and gastric adenocarcinoma with hepatitis B carrier status. We treated the patient with 8 courses of pirarubicin bleomycin, vincristine, and dacarbazine (modified ABVD), and 4 courses of capecitabine therapy concurrently along with oral entecavir, as the patient survived longer than 20 months.The prognosis of multiple primary malignancies is poor because therapy is difficult, without a standard treatment. The frequency of multiple primary malignancies is increasing in recent years, and second malignancies in patients with cancer should be taken into consideration.

Outcome and prognosis of spinal myeloma surgery.