Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 58-year-old male diagnosed as having primary myelofibrosis with thrombocytopenia, who died of fatal septic shock and rhabdomyolysis after platelet concentrates (PCs) transfusion. The initial diagnosis of primary myelofibrosis was established by splenomegaly, leukoerythroblastosis and bone marrow fibrosis. PCs were transfused because of thrombocytopenia with marked bleeding tendency. Soon after the PCs transfusion in March 2000, he had attacks of chest pain, back pain and myalgia, then went into shock and died of unknown causes. PCs were suspected as being the cause of death, because Streptococcus pneumoniae was found in the culture of PCs in the WBC-reduction in-line filter and fresh frozen plasma from the same donor preserved in the Japan Red Cross Center. Rhabdomyolysis, neutrophil infiltration and phagocytosed bacteria were found from the autopsy materials, which were identified by DNA analysis as the same species found in the PCs. PCs are kept at room temperature because platelet function is lost in the cold. When PCs are contaminated with bacteria, marked multiplication induces fatal bacteremia. This is a rare report in Japan of fatal septic shock caused by PCs with bacterial contamination. We must pay strict attention to bacterial contamination in blood components.
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PMID:[Fatal septic shock and rhabdomyolysis following transfusion of platelet concentrates contaminated with Streptococcus pneumoniae]. 1288 16

The case of a 65 year old woman referred for further evaluation of back pain and with abnormalities at ultrasound including increase in portal vein diameter and splenomegaly is presented. Other tests, including bone marrow biopsy and Doppler ultrasound, led to a diagnosis of portal vein thrombosis secondary to chronic myeloid leukemia. After prompt cytoreductive therapy with leukapheresis and hydroxyurea, resolution of portal vein thrombosis and portal hypertension was achieved within a in one-month period. An abnormal increase of cells in circulating blood may lead to portal vein thrombosis in patients with myeloproliferative disorders such as chronic myeloid leukemia. Chronic myeloid leukemia is an unusual cause of portal vein thrombosis and portal hypertension. Early administration of cytoreductive therapy may lead to the resolution of portal vein thrombosis. In this report, etiopathogenetic factors of portal vein thrombosis and the role of cytoreductive therapy in the dissolution of thrombosis are discussed.
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PMID:Rapid resolution of portal vein thrombosis and noncirrhotic portal hypertension following cyto-reductive therapy in a patient with chronic myeloid leukemia. 1461 43

Chronic Neutrophilic Leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of Philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of Polycythemia Vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2 cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91,800/microL (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1,661,000/microL. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M:E ratio of 10:1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no Philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40,000/microL.
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PMID:A case of transition of polycythemia vera to chronic neutrophilic leukemia. 1568 21

Dengue fever (DF) and Dengue hemorrhagic fever (DHF) are widespread in Southeast Asia. An outbreak of DF/DHF in Delhi in 2003 started during September, reached its peak in October-November, and lasted until early December. This study describes the clinical and laboratory data of the 185 cases of DF/DHF admitted to Lok Nayak Hospital, New Delhi. The mean age of the patients was 26 +/- 10 years. Fever was present in all the cases with an average duration of fever being 4.5 +/- 1.2 days with headache (61.6%), backache, (57.8%), vomiting (50.8%) and abdominal pain (21%) being the other presenting complaints. Hemorrhagic manifestations in the form of a positive tourniquet test (21%), gum bleeding and epistaxis (40%), hematemesis (22%), skin rashes (20%) and melena (14%) were also observed. Hepatomegaly and splenomegaly were observed in 10% and 5% of cases, respectively. Laboratory investigations revealed thrombocytopenia (with a platelet count of < 100,000/microl) in about 61.39% of cases, Leukopenia (WBC <3,000/mm2) and hemoconcentration (Hct >20% of expected for age and sex) were found in 68% and 52% of the cases, respectively. The mortality rate was 2.7%. Despite widespread measures taken to control outbreaks of DF, it caused major outbreaks. More stringent measures in the form of vector control, improved sanitation and health education are needed to decrease morbidity, mortality and health care costs caused by a preventable disease.
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PMID:The 2003 outbreak of Dengue fever in Delhi, India. 1643 42

Patients with human immunodeficiency virus (HIV) infection are at increased risk for developing lymphoproliferative disorders. Multicentric Castleman's disease should always be kept in the differential diagnosis of HIV-positive patients suspected of having lymphoma to avoid misdiagnosis. We report the case of a 40-year-old HIV-positive homosexual man who presented with lower back pain and features highly suggestive of lymphoma including lymphadenopathy, elevated lactic dehydrogenase, and splenomegaly. The patient's plasma was positive by polymerase chain reaction for Kaposi sarcoma herpesvirus/human herpesvirus-8 (KSHV/ HHV8), and a lymph node biopsy revealed Multicentric Castleman's disease. He was started on highly active antiretroviral therapy, corticosteroids, valganciclovir, and rituximab. His back pain subsided, lymphadenopathy regressed, and he became KSHV/HHV8 negative. Albeit a rare condition, Castleman's disease should always be considered in immunocompromised patients suspected of having lymphoma.
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PMID:Multicentric Castleman's disease masquerading as HIV-related lymphoma. 1803 Jan 93

A 48-year old man presented with back pain that was resistant to treatment. An MR scan showed spondylolisthesis at L4-5 and narrowing of the exit foraminae. He had a posterior fusion which did not relieve his symptoms. He continued to have back pain and developed subcutaneous nodules in both forearms. Biopsy from the skin revealed cutaneous sarcoidosis, and one from the lumbar spine showed sarcoidosis granuloma between the bone trabeculae. A CT scan of the abdomen and chest revealed axillary lymphadenopathy, mediastinal enlarged nodes, apical nodular nodes and splenomegaly. The patient was started on large doses of methotrexate and steroids. His angiotensin-converting enzyme and calcium levels returned to normal and the back pain resolved.
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PMID:A rare presentation of sarcoidosis, back pain and spondylolisthesis. 1825 97

The authors report on a 54-year-old man who presented with a lumbar vertebral body lesion and an adjacent epidural lesion that was found to be hairy cell leukemia (HCL). The patient presented with gradual onset of back pain and intermittent lower-extremity radicular symptoms. He did not have splenomegaly or peripheral blood count abnormalities. Admission MR imaging revealed an L-5 vertebral body lesion and a lumbar epidural lesion extending from L-3 to S-2. An [18F]fluorodeoxyglucose-PET study showed numerous sites of osseous involvement. The patient underwent minimally invasive surgical biopsy sampling of the epidural lesion. Histopathological examination revealed extranodal HCL. After treatment with a 5-day course of cladribine, the patient's symptoms resolved, and at the 16-week follow-up visit there was no radiographic or metabolic evidence of disease. Hairy cell leukemia rarely involves neurological structures, but this patient responded well to standard treatment. This case demonstrates the value of tissue biopsy procedures instead of aggressive resection and the use of minimally invasive techniques to treat an HCL spinal lesion.
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PMID:Extranodal hairy cell leukemia presenting in the lumbar spine. 1893 25

Acute splenic sequestration crisis is a common, potentially life-threatening complication of sickle cell anemia in children that is uncommon in adults.We present the case of a 44-year-old gentleman with undiagnosed hemoglobin S-C disease who developed intense back pain, marked abdominal distension, systemic inflammatory response syndrome, and multisystem organ failure that first presented as acute splenic sequestration crisis. The hemoglobinopathy SC is a disease caused by heterozygous-globin chain mutations with over-lapping clinical features of sickle cell disease with changes in the frequency of these manifestations reflected by the combination of characteristics of hemoglobin C and hemoglobin S. In hemoglobin S-C disease, autosplenectomy is rare and splenomegaly usually persists until adulthood;vasoocclusive complications are seen less habitually and become evident at a later time compared with sickle cell disease. The diagnosis of hemoglobin S-C disease is essentially done by exclusion. Transfusion of red blood cells is the treatment of choice, but splenectomy is indicated if transfusion therapy fails. A review of the literature and keypoints for the emergency practitioner are included.
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PMID:Acute splenic sequestration in an adult with hemoglobin S-C disease. 1909 Dec 80

Central nervous system involvement is a serious complication of brucellosis; data found in the literature are generally restricted to case reports and case series. In this study we pooled the data from Turkish medical practice in order to gain a thorough understanding of the subject. A search of Turkish publications on chronic neurobrucellosis was made using both Turkish and international databases. A total of 35 publications were analyzed and 187 neurobrucellosis cases were evaluated. Headache, fever, sweating, weight loss, and back pain were the predominant symptoms, while meningeal irritation, confusion, hepatomegaly, hypoesthesia, and splenomegaly were the most frequent findings. The major complications in patients were cranial nerve involvement, polyneuropathy/radiculopathy, depression, paraplegia, stroke, and abscess formation. Antibiotics were used in different combinations and over different intervals. The duration of antibiotic therapy reported ranged from 2 to 15 months (median 5 months). The mortality was 0.5% with suitable antibiotics. Neurobrucellosis may mimic various pathologies. For this reason, a thorough evaluation of the patient with probable disease is crucial for an accurate diagnosis and proper management of the disease.
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PMID:Overview of neurobrucellosis: a pooled analysis of 187 cases. 1942 83

Rupture of the spleen is a potentially life-threatening condition, which most often occurs secondary to abdominal trauma. Spontaneous rupture of the spleen is a much rarer event, usually occurring secondary to infections and less frequently secondary to haematological malignancies causing massive splenomegaly. We present a case of a 71-year-old woman who presented in the emergency department with acute abdominal and back pain and no history of trauma, with a CT scan diagnosis of splenic rupture. Splenectomy was performed and the histological examination of the specimen revealed splenic marginal cell lymphoma (SMZL), which is classified under the non-Hodgkins lymphomas (NHL) and accounts for <1% of NHL. There is only one previously reported case of spontaneous splenic rupture SMZL and this is the first recorded case of spontaneous splenic rupture in a patient without massive splenomegaly.
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PMID:Splenic marginal cell lymphoma (SMZL): report of its presentation with spontaneous rupture of the spleen. 2496 36


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