Classical angiomyolipomas are benign tumors composed of various tissues, including fat, abnormal blood vessels and smooth muscle cells. The present study reports a male patient affected by mediastinal angiomyolipomas with massive chylous pleural effusion. The tumors were characterized with histological and immunohistochemical methods.
Angiomyolipoma ; complications ; Humans ; Male ; Mediastinum ; pathology ; Middle Aged ; Pleural Effusion ; diagnosis ; etiology

Pleural effusion in chronic myeloid leukemia (CML) is poorly understood and rarely reported in the literature. When the pleural effusion is caused by leukemic pleural infiltration, the differential white blood cell count of the effusion is identical to that of the peripheral blood, and the fluid cytology reveals leukemic blasts. We report here a case of bilateral pleural involvement of atypical CML in an 83-yr old male diagnosed with pancreatic cancer with abdominal wall metastasis and incidental peripheral leukocytosis. Based on bone marrow examination, chromosome analysis and polymerase chain reaction he was diagnosed with Philadelphia chromosome negative, BCR/ABL gene rearrangement negative CML. Following 3 months of treatment with gemcitabine for pancreatic cancer, he developed bilateral pleural effusions. All stages of granulocytes and a few blasts were present in both the pleural fluid and a peripheral blood smear. After treatment with hydroxyurea and pleurodesis, the pleural effusion resolved.
Pleural Effusion/*etiology/pathology ; Male ; Leukemic Infiltration/*pathology ; Leukemia, Myeloid, Chronic/complications/*pathology ; Humans ; Aged, 80 and over ; Aged

Animals ; Arteriovenous Malformations ; complications ; Child, Preschool ; Diaphragm ; blood supply ; Hemothorax ; etiology ; Humans ; Male ; Nematode Infections ; pathology ; Pleural Effusion ; etiology

OBJECTIVETo analyze the clinical characteristics and diagnosis of 2 cases with chylothorax due to primary lymphatic dysplasia and to elevate pediatrician's recognition level for this disease.

METHODClinical manifestations of the children were retrospectively analyzed. Primary lymphatic dysplasia was diagnosed by lymphoscintigraphy.

RESULTThe first patient was a male aged 2-year-7-month who presented with a history of tachypnea for 43 days, fever and sore throat for 5 days at the early stage of the illness. He had a history of external injury before his illness. Physical examination showed his left chest bulging and left side diminished breath sound. His pleural effusion showed dark red (It was divided into two layers after standing, the upper layer turned into milky white, and the lower turned into hemorrhagic liquid) . White blood cell (WBC) count was 9 000×10(6)/L, mononuclear cell was 0.9, polykaryocytes was 0.1, triglyceride was 12.37 mmol/L in the pleural effusion. Contrast-enhanced lung CT (revascularization) showed pericardial effusion and a massive left sided pleural effusion. The second patient was a male aged 9 years and 6 months, who presented with a history of cough for 24 days, intermittent fever, vomiting, abdominal pain for 19 days, and edema of lower limbs for 4 days. Physical examination showed edema in both eyelids, lower extremities and scrotum. The level of albumin was 14 g/L and the titer of Mycoplasma pneumoniae IgM was 1: 320 in the serum. His hydrothorax pleural effusion showed milk white. White blood cell (WBC) count was 74×10(6)/L, mononuclear cell was 0.78, polykaryocytes was 0.22, triglyceride was 1.01 mmol/L in the pleural effusion. Chyle test showed positive in his pleural effusion and seroperitoneum. High-resolution CT of the lung revealed bilateral interstitial and parenchymal infiltration and both sided pleural effusion. Abdominal ultrasound showed giant hypertrophy of the gastric mucosa and massive ascites. Gastroscopy showed giant hypertrophy of the gastric mucosa. Lymphoscintigraphy revealed primary lymphatic dysplasia in both children.

CONCLUSIONPrimary lymphatic dysplasia might occur in children and result in dropsy of serous cavity (chylothorax, chylopericardium, chylous ascites). Dropsy of serous cavity showed bloody or milk white. WBC count might elevate with lymphocyte increasing mostly, triglyceride was often higher than 1.0 mmol/L in dropsy of serous cavity. Primary lymphatic dysplasia can be diagnosed by lymphoscintigraphy.

Child ; Child, Preschool ; Chylothorax ; diagnosis ; etiology ; pathology ; Humans ; Leukocyte Count ; Lymphatic Abnormalities ; complications ; diagnosis ; pathology ; Lymphoscintigraphy ; Male ; Pericardial Effusion ; diagnosis ; etiology ; Pleural Effusion ; diagnosis ; etiology ; pathology ; Tomography, X-Ray Computed

While pleural effusion in multiple myeloma is relatively infrequent, myelomatous pleural effusion is extremely rare. We experienced a 61-year-old woman with IgD-lambda multiple myeloma and pleural effusion. The diagnosis was made originally by pleural biopsy, pleural fluid cytology and immunoelectropheresis of pleural fluid. Transient improvement of the pleural effusion was observed after administration of combination chemotherapy of vincristine, melphalan, cyclophosphamide, prednisone (VMCP)/vincristine, cyclophosphamide, adriamycin, prednisone (VCAP). Two months later, myelomatous pleural effusion recurred and no response to salvage therapy was observed. We reviewed the clinical feature of this case and literature concerning myelomatous pleural effusion.
Antineoplastic Agents, Combined/administration & dosage* ; Case Report ; Cyclophosphamide/administration & dosage ; Female ; Human ; Melphalan/administration & dosage ; Middle Age ; Multiple Myeloma/pathology ; Multiple Myeloma/drug therapy* ; Multiple Myeloma/complications* ; Plasma Cells/pathology ; Pleural Effusion/radiography ; Pleural Effusion/pathology ; Pleural Effusion/etiology* ; Prednisone/administration & dosage ; Tomography, X-Ray Computed ; Vincristine/administration & dosage

While pleural effusion in multiple myeloma is relatively infrequent, myelomatous pleural effusion is extremely rare. We experienced a 61-year-old woman with IgD-lambda multiple myeloma and pleural effusion. The diagnosis was made originally by pleural biopsy, pleural fluid cytology and immunoelectropheresis of pleural fluid. Transient improvement of the pleural effusion was observed after administration of combination chemotherapy of vincristine, melphalan, cyclophosphamide, prednisone (VMCP)/vincristine, cyclophosphamide, adriamycin, prednisone (VCAP). Two months later, myelomatous pleural effusion recurred and no response to salvage therapy was observed. We reviewed the clinical feature of this case and literature concerning myelomatous pleural effusion.
Antineoplastic Agents, Combined/administration & dosage* ; Case Report ; Cyclophosphamide/administration & dosage ; Female ; Human ; Melphalan/administration & dosage ; Middle Age ; Multiple Myeloma/pathology ; Multiple Myeloma/drug therapy* ; Multiple Myeloma/complications* ; Plasma Cells/pathology ; Pleural Effusion/radiography ; Pleural Effusion/pathology ; Pleural Effusion/etiology* ; Prednisone/administration & dosage ; Tomography, X-Ray Computed ; Vincristine/administration & dosage

Pleuropulmonary blastoma (PPB) is a rare dysontogenetic neoplasm in children and has been recognized as a distinct clinicopathological entity different from the ordinary pulmonary blastoma of adulthood. We report a very rare adult case of PPB. A 43-year-old female patient presented with massive pleural infusion, and a misdiagnosis of pleural tuberculosis (TB) was made on the basis of ultrasound scan and CT images, for which therapy with TB drugs was administered for 3 weeks. Subsequent operation and pathologic examination of the mass revealed a multicystic neoplasm consisting of malignant mesenchymal cells immunohistochemically positive for vimentin and actin. Local recurrence occurred in the left pleural 20 months after the surgical tumor resection and 4 cycles of adjuvant chemotherapy.
Adult ; Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms ; diagnosis ; pathology ; surgery ; Pleural Effusion ; diagnosis ; etiology ; Pulmonary Blastoma ; diagnosis ; pathology ; surgery ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Intra-abdominal hypertension (IAH) is being increasingly reported in patients with severe acute pancreatitis (SAP) with worsened outcomes. The present study was undertaken to evaluate intra-abdominal pressure (IAP) as a marker of severity in the entire spectrum of acute pancreatitis and to ascertain the relationship between IAP and development of complications in patients with SAP. METHODS: IAP was measured via the transvesical route by measurements performed at admission, once after controlling pain and then every 4 hours. Data were collected on the length of the hospital stay, the development of systemic inflammatory response syndrome (SIRS), multiorgan failure, the extent of necrosis, the presence of infection, pleural effusion, and mortality. RESULTS: In total, 40 patients were enrolled and followed up for 30 days. The development of IAH was exclusively associated with SAP with an APACHE II score > or =8 and/or persistent SIRS, identifying all patients who were going to develop abdominal compartment syndrome (ACS). The presence of ACS was associated with a significantly increased extent of pancreatic necrosis, multiple organ failure, and mortality. The mean admission IAP value did not differ significantly from the value obtained after pain control or the maximum IAP measured in the first 5 days. CONCLUSIONS: IAH is reliable marker of severe disease, and patients who manifest organ failure, persistent SIRS, or an Acute Physiology and Chronic health Evaluation II score > or =8 should be offered IAP surveillance. Severe pancreatitis is not a homogenous entity.
APACHE ; Acute Disease ; Adult ; Female ; Humans ; Intra-Abdominal Hypertension/*etiology ; Length of Stay ; Male ; Middle Aged ; Multiple Organ Failure/etiology ; Necrosis/etiology ; Pancreas/*pathology ; Pancreatitis/*complications/mortality/physiopathology ; Pleural Effusion/etiology ; Prospective Studies ; Severity of Illness Index ; Systemic Inflammatory Response Syndrome/etiology

OBJECTIVETo study the clinical characteristics of Streptococcus pneumonia-associated hemolytic uremic syndrome (SP-HUS) in children.

METHODClinical and laboratory data of a pediatric case of SP-HUS were retrospectively analyzed and the key points of diagnosis and therapy were reviewed.

RESULTAn 18-month old girl was admitted with chief complaint of fever and cough for 5 days combined with mild labored breath. Breath sound was found weakened in right lung with lower lobe dullness on percussion. Laboratory tests revealed: WBC 3.7×10(9)/L, Hb 83 g/L, PLT 11×10(9)/L, C-reactive protein (CRP) > 180 mg/L. Morphological study of the RBCs showed marked anisocytosis and schistocytosis. Urinalysis showed 42.66 RBCs per high-power field, occult blood (+++), proteinura (++++). Streptococcus pneumoniae was isolated from blood, pleural fluid and sputum. Serotyping with simplified chessboard system was 3. The direct Coombs test was positive. Serum complement levels (C3 and C4) were depressed at 0.699 g/L, 0.064 g/L, respectively. Chest X-ray showed pleural effusion and infection of the right hemothorax. The computerized tomographic scan of the chest revealed pneumatoceles in the right lower lobe. The diagnosis on admission we considered was SP-HUS. Intravenous antibiotic therapy (vancomycin + cefoperazone/sulbactam) was administered. The renal replacement theraphy was administered to maintain electrolyte and fluid balances and adequate nutrition. Transfusions of washed red blood cells were administered to correct the anemia. One month after admission the patient was good with recovery. Liver and renal function recovered and the pneumonia was resolving, anemia and platelets were corrected. The direct Coombs test turned to be negative. Serum complement levels (C3 and C4) were normal. After 3-month follow-up, no clinical anomalies were detected.

CONCLUSIONSP-HUS should be suspected when the following occurs in the context of pneumococcal infections: microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure and a positive Coombs test result. Serotype 3 of SP was associated with HUS.

Anti-Bacterial Agents ; therapeutic use ; Biomarkers ; analysis ; Coombs Test ; Female ; Hemolytic-Uremic Syndrome ; diagnosis ; etiology ; microbiology ; therapy ; Humans ; Infant ; Lung ; diagnostic imaging ; pathology ; Pleural Effusion ; etiology ; Pneumococcal Infections ; complications ; Radiography ; Retrospective Studies ; Serotyping ; Streptococcus pneumoniae ; classification ; isolation & purification

A 60-year-old man presented with cough, sputum, and dyspnea. He had a history of acute myeloid leukemia and hematopoietic stem cell transplantation with chronic renal failure. Chest CT scans showed miliary nodules and patchy consolidations. Histological examination revealed numerous fibrin balls within the alveoli and thickening of the alveolar septum, both of which are typical pathological features of acute fibrinous and organizing pneumonia (AFOP). We report the first case of AFOP following allogeneic hematopoietic stem cell transplantation.
Acute Disease ; Anti-Bacterial Agents/therapeutic use ; Biopsy ; Cryptogenic Organizing Pneumonia/etiology/pathology ; Fatal Outcome ; Glucocorticoids/administration & dosage ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Hemoptysis/etiology ; Humans ; Leukemia, Myeloid, Acute/*surgery ; Lung Diseases/*etiology/pathology ; Male ; Middle Aged ; Pleural Effusion/etiology ; Pulse Therapy, Drug ; Radiography, Thoracic ; Respiratory Insufficiency/etiology ; Tomography, X-Ray Computed