Cases reported "Splenomegaly"

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1/22. Spontaneous (pathological) splenic rupture in a blastic variant of mantle cell lymphoma: a case report and literature review.

    Spontaneous (pathological) splenic rupture (SPSR) in hematological malignancies is rare. This report describes a 71-year-old male diagnosed with mantle cell lymphoma-blastic variant (MCL-BV) who experienced an SPSR a few days before the initial diagnosis. The patient underwent a splenectomy and recovered without incident. Partial remission was seen following several cycles of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone). However, relapse was rapid, with leukemic meningitis occurring several months later. It was successfully treated by intrathecal methotrexate and cranial spinal radiation. A progressive lymphocytosis developed, which responded to rituximab. Lymphadenopathy and skin involvement ensued, followed by pneumonia and death. The literature on SPSR in patients with MCL-BV and other lymphoproliferative disorders showed similar clinical and postoperative findings. Clinical presentation included Kehr's sign and acute abdominal pain. Postoperative findings included blood in the peritoneal cavity, multiple splenic hematomas, splenic infarcts, and splenic necrosis. Most strikingly, the majority of the patients reviewed appeared to have undergone some type of blastic transformation. One or any combination of these findings that has been noted above in addition to a bleeding diathesis could be the foundation to SPSR. We recommend consideration of splenic rupture in patients with a lymphoproliferative disorder coupled with rapid progression of marked or massive splenomegaly.
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2/22. Idiopathic myelofibrosis associated with classic polyarteritis nodosa.

    A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.
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3/22. Primary abdominal lymphonodular cryptococcosis in children: 2 case reports and a literature review.

    BACKGROUND: cryptococcus neoformans is an important opportunistic fungal pathogen that is acquired via the respiratory tract. It causes several clinical syndromes and most commonly presents as meningitis. The establishment of C neoformans infections in immunocompetent individuals could be owing to increased virulence, dose of the organism, or genetically determined differences in the ability to generate a protective immune response against the organism. OBJECTIVES: The purpose of this study is to report on 2 case studies and a literature review and to discuss the diagnosis and management of primary abdominal lymphonodular cryptococcosis. methods: We conducted 2 case studies and a literature review. RESULTS: Two cases of primary abdominal lymphonodular cryptococcosis in childhood were reported herein. The patients suffered from C neoformans without immunoglobulin or lymphocyte abnormalities. The cause of hospitalization of the 2 cases was mainly the persisting fever with or without abdominal pain. Imaging findings indicated dramatic lymphadenopathy and light hepatosplenomegaly. Excision biopsy of the abnormal lymph node demonstrated reactive lymphoid hyperplasia and the presence of C neoformans. The results of nested polymerase chain reaction confirmed the diagnosis of C neoformans. CONCLUSION: This is the first report on C neoformans in the primary abdominal lymph node in children. This report suggests that C neoformans infection should be considered in the differential diagnosis of fever and marked lymphadenopathy, and empirical administration of antifungal agent may be necessary. C neoformans antibody test, polymerase chain reaction assay, fine-needle aspiration biopsy, or surgical excision biopsy is needed in early diagnosis.
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4/22. Stauffer's syndrome variant with cholestatic jaundice: a case report.

    cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer's syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, alpha-2-globulin, and gamma-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer's syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.
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5/22. Acute portal vein thrombosis and massive necrosis of the liver. An unusual complication after stenting for chronic pancreatitis.

    CONTEXT: ERCP can provide information which is invaluable in managing chronic pancreatitis but it is associated with infrequent, although significant, complications and rare mortality. The complications uniquely associated with diagnostic ERCP include pancreatitis and sepsis (primary cholangitis). CASE REPORT: A 32-year-old man presented with severe upper abdominal pain radiating to the back, associated with vomiting and abdominal distension. The patient was diagnosed as having had chronic calcific pancreatitis recently and had undergone ERCP with pancreatic duct stenting elsewhere. Two days after the procedure, the patient developed severe abdominal pain, vomiting and abdominal distention, and patient was referred to our hospital 7 days after the procedure. Investigation revealed massive liver necrosis and portal vein thrombosis. This patient had a life-threatening complication following pancreatic duct stenting for chronic pancreatitis and was managed medically. CONCLUSION: Therapeutic pancreatic endoscopy procedures are technically demanding and should be restricted to high volume centers. There is a continuing need for evaluation and comparison with alternative strategies. In a good surgical candidate, it is better to avoid stenting.
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6/22. Preeminence of lesser splanchnic blood flow in selected patients with generalized portal hypertension.

    Although restricted transhepatic portal flow is necessary for development of generalized portal hypertension (GPH), increased splanchnic arterial inflow also contributes to GPH and its clinical sequelae. In this context, we describe 7 male and 6 female patients (mean age 48 years) in whom the lesser splanchnic (gastrosplenic) system played a key role in the signs and symptoms of GPH. These 13 patients (9 with hepatic cirrhosis, 3 with primary myeloproliferative disorder, and 1 with extrahepatic portal block) shared common features of massive splenomegaly, huge splenofundic gastric varices, often with a prominent natural shunt to the left renal vein. Total or near total splenectomy alone or combined where appropriate with coronary vein ligation was effective in controlling varix hemorrhage (10 patients), ascites (3), or complications of an enlarged spleen-anorexia and abdominal pain (3), hemolytic anemia (1) and profound thrombocytopenia with severe epistaxis (1). Intraoperative jejunal portal venography was crucial in operative management in order to establish definitively the presence or absence of coronary venous collaterals, and when present, to verify their operative ligation. These distinctive patients illustrate: 1) GPH is a heterogeneous syndrome of divergent splanchnic circulatory patterns, a feature which should be taken into account in selecting operative treatment; 2) one well-defined subgroup displays prominent hyperdynamic lesser splanchnic and specifically, splenic blood flow as a major contributor to clinical complications; and 3) within this subgroup, splenectomy combined with documented absence or surgical interruption of coronary venous collaterals as corroborated by intraoperative portography is effective alternative treatment.
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7/22. splenic rupture and infectious mononucleosis.

    infectious mononucleosis is an acute viral illness associated with a high incidence of splenomegaly, although the incidence of splenic rupture is low. When rupture occurs, the mortality has been significant, presumably, because a history of trauma is not present. The spleen may be vulnerable to injury due to the histopathologic changes that occur as a result of this illness. Essentially all patients with spontaneous rupture related to infectious mononucleosis have epigastric or upper abdominal pain. The diagnosis of splenic rupture may be confirmed in a variety of ways. In those patients who are hemodynamically stable, CT scan, ultrasound, or radionuclide scan may aid in establishing the diagnosis. Selective splenic angiography is very accurate but has been largely abandoned because of the invasive nature of the study. peritoneal lavage is efficacious in establishing the diagnosis in hemodynamically unstable patients. The treatment of choice, at this time, is splenectomy. Current interest in splenic salvage has resulted in reports of nonoperative therapy in stable patients and splenorrhaphy in one instance. Due to the extent of the histologic changes in the spleen, caution is urged in electing the conservative approach to this clinical situation.
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8/22. Hepatic sinusoidal dilatation with portal hypertension during azathioprine treatment after kidney transplantation.

    An unusual hepatic disease developed in 3 patients with a well-functioning kidney graft 16-24 months after transplantation. Vague abdominal pain, increased bleeding tendency and edema were initial complaints, and hepato- or splenomegaly and ascites were found as well. liver function tests were not or only mildly disturbed; hemolysis and pancytopenia were always present. Colloid uptake was absent at liver scintigraphy and the hepatic venous wedge pressure was increased. Esophageal varices were demonstrated. Liver biopsy showed extensive midzonal and pericentral sinusoidal dilatation. After discontinuation of azathioprine the symptoms and the extent of sinusoidal dilatation disappeared gradually, but after 1-3 years fibrosis or micronodular cirrhosis had developed and splenomegaly with hypersplenism remained. These observations strongly suggest an association between chronic use of azathioprine and the development of venous congestion of the liver with sinusoidal dilatation, eventually resulting in chronic liver disease.
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9/22. Rectus sheath hematoma mimicking acute splenic disease.

    In patients with hematological malignancies and splenomegaly, acute abdominal pain in the left upper quadrant is highly suspicious of splenic disease (i.e., hematoma, infarction, or rupture). We report the case of a patient with chronic myelogenous leukemia and splenomegaly who presented an unusual abdominal condition causing pain in the left upper quadrant, with a clinical presentation mimicking acute splenic disease. The diagnostic dilemma was resolved by ultrasonography, demonstrating a rectus sheath hematoma.
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10/22. Splenic torsion. An unusual cause of splenomegaly.

    A 28-year-old woman who presented with acute abdominal pain and splenomegaly was found to have little functioning splenic tissue on liver-spleen scan but a large homogeneous spleen by computerized axial tomography. laparotomy revealed torsion of the splenic pedicle and extensive splenic infarction. The presentation, pathophysiology, and diagnostic difficulties of this case are discussed. Splenic torsion should be considered in the differential diagnosis of painful splenomegaly.
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