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

The onset of postpolycythemic myeoloid metaplasia or spent polycythemia has been recognized for many years. As the result of many different series, the development of postpolycythemic myeolid metaplasia might be expected in from 15%-20% of patients with postpolycythemia vera. It appears that an etiologic role for sodium phosphate 32P may exist in this evolutionary pattern. About 70% of patients with PPMM will have symptoms with the onset of the syndrome. The major mechanisms producing symptoms result from (1) anemia, (2) pressure from massive splenomegaly, and (3) bleeding problems. Iron deficiency is a frequent cause of anemia in patients with PPMM. The major mechanism of anemia in these patients, however, relates to ineffective erythropoiesis and shortened red cell survival. Androgen trials for ineffective erythropoiesis seem worthwhile, although data on this point is too limited to draw any firm conclusions. A steroid trial for those patients with major hemolytic episodes is indicated. In those patients in whom adrenal steroid therapy fails to control major hemolysis, a consideration for splenectomy exists. Pressure-related manifestations secondary to massive splenomegaly have been treated with radiation therapy and oral alkylators. Although there is data to document amelioration of painful symptoms with associated shrinking of the spleen, long-term control of this problem has not been forthcoming. Again, patients who are medical failures in control of pressure-related manifestations may be considered for splenectomy. Bleeding problems may arise with PPMM secondary to thrombocytopenia, thrombocythemia, or qualitative platelet dysfunction. Adrenal steroids have met with some success in improving platelet counts in patients with life-threatening thrombocytopenia. Those patients who are medical failures with adrenal steroids in terms of thrombocytopenia might be candidates for splenectomy. Control of thrombocythemia has been observed with oral alkylator therapy and chlorambucil may have a special role in managing this complication. Qualitative platelet defects leading to severe bleeding are best managed with fresh platelet transfusions. Patients with PPMM in contrast to patients with agnogenic myeoloid metaplasia have a more lethal syndrome and shortened survivorship. Causes of death in patients with PPMM include cardiac problems, transition to acute leukemia, hemorrhage, and infection.
 ...
PMID:The evolution into and the treatment of late stage polycythemia vera. 125 Dec 24

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
 ...
PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

We report a case of adrenal extramedullary hematopoiesis in a 27-year-old woman with beta-thalassemia. She underwent cholecystectomy for gallstones and splenectomy for splenomegaly at the age of 20 years. In January 1996, she was admitted to our hospital because of a palpable nontender mass in the right subcostal area. Ultrasound, computed tomography, and magnetic resonance imaging disclosed a right suprarenal mass 7.5 x 5.8 cm in size. Surgical exploration and biopsy confirmed the diagnosis of extramedullary hematopoietic (EMH) tumor. The tumor was left in situ. Subsequent follow-up showed no increase in the size of the tumor and no progression of anemia. The patient remained asymptomatic, and no additional EMH tumor developed. Surgery or radiotherapy should be performed only when EMH tumors cause symptoms, such as during spinal cord compression. Adrenal EMH tumors should be considered in thalassemia patients with an adrenal mass, to avoid unnecessary surgical procedures.
 ...
PMID:Adrenal extramedullary hematopoietic tumor in a patient with beta-thalassemia. 965 Apr 75

Body, adrenal, brain, heart, liver, kidney, spleen and testis masses were determined for agouti and non-agouti deer mice (Peromyscus maniculatus gracilis) of both sexes. Body mass was highest for non-agouti females and lowest for agouti females; and sex differences in body mass were significant for agouti, but not non-agouti, deer mice. Adrenal, brain and liver masses were similar between color morphs; heart mass was greater in agouti males; and kidney, spleen and testis masses were all significantly greater for non-agouti deer mice. Splenomegaly in non-agouti deer mice was prominent, as spleens of non-agouti deer mice were 50% larger than those of agouti animals. Sex differences varied across organs and color morphs. For both color morphs, males had heavier adrenals and brains, whereas females had heavier livers and spleens. Kidney and heart mass was greater for female non-agouti deer mice, but for agouti animals, heart mass was greater in males and kidney mass differed little between the sexes. For both color morphs, testes and spleen mass was altered by photoperiod in 72 deer mice housed under short- or long-day conditions and the effect was stronger in non-agouti animals. This is the first report of splenomegaly and sex-specific body mass differences associated with the non-agouti allele.
 ...
PMID:Body and organ mass in agouti and non-agouti deer mice (Peromyscus maniculatus). 1154 76

A 9-year-old boy was admitted to Xiangya Hospital due to pain after trauma in the left lower limb for 5 days and fever with generalized pain for 2 days. The results of X-ray of the left lower limb were normal. Pulmonary computed tomography (CT) showed multiple pulmonary nodules in both lungs. Adrenal CT showed marked enlargement of the left adrenal gland. The patient also experienced generalized herpes and intermittent delirium and had a blood pressure up to 155/93 mm Hg. He was transferred to our hospital with a suspected diagnosis of pheochromocytoma. On admission, the patient had a blood pressure of 86/44 mm Hg, sporadic maculopapule and herpes, touch-evoked pain, exposure of superficial veins, white pus coating on the right side of the tongue, and tension in the abdominal muscle. No skin damage was observed in the left lower limb, and the patient was forced to be in the extending position and experienced significant swelling below the knees. Laboratory examination showed a reduction in platelet count, hypoproteinemia, a significant increase in creatase, a C-reactive protein level of 348 mg/L, and a procalcitonin level of >100 ng/mL. Thoracoabdominal and pelvic CT showed multiple patchy and nodular lesions in both lungs, which had an undetermined nature, as well as an enlarged spleen. The tests of puncture fluid from the left knee joint and the periosteum of the left tibia, blood culture, and bone marrow culture all showed methicillin-resistant Staphylococcus aureus. The patient was given anti-shock treatment, anti-infective therapy with vancomycin, debridement and continuous irrigation/drainage of osteomyelitis lesions in the left tibia, but the patient still experienced recurrent shivering and severe fever and increased subcutaneous and pulmonary nodules. Linezolid was added on day 8 after admission, and the patient's body temperature returned to normal on day 24 after admission. Subcutaneous and pulmonary nodules were gradually reduced and disappeared. The patient was treated for 2 months and then evaluated as cured.
 ...
PMID:[Fever, generalized pain, and multiple pulmonary nodules in a school-aged boy]. 2765 44

Cirrhosis has many complications regardless of the aetiology. Complications include splenomegaly, ascites, hepatic encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome and hepatocellular carcinoma and also linked to abnormalities in the endocrine system, including abnormal sex hormone metabolism, thyroid disease, osteoporosis, and, most recently identified, adrenal insufficiency. This prospective cohort study was done to evaluate the impact of adrenocortical insufficiency on clinical parameters in haemodynamically stable cirrhotic patients with ascites and had been performed at the inpatient of GHPD Department, BIRDEM, Dhaka, Bangladesh from April 2011 to March 2012. A total of fifty three (53) patients fulfilling inclusion criteria were included in the study. Patients were divided into two groups: Group A (patients of normal adrenal function) and Group B (patients of insufficient adrenal function) and those were followed up for the next 6 months. In Group A, the total number of patients was 25(47%) and in Group B it was 28(53%). Between two groups, mean age difference and gender difference were not statistically significant (p value was 0.278 and 0.933, respectively). Group B patients had significant higher CLD duration (p=0.004). Haematemesis and/or maelena was significantly lower in Group B at follow up (p=0.0001) due to significant higher number of band ligation in this group (p=0.009). Hepatic encephalopathy was significantly higher in Group B at enrollment (p=0.028) and at follow up (p<0.001). During the period of follow up, significant higher number of patients had developed hepatic encephalopathy in Group B compared to Group A (p<0.05). There was statistically significant higher number of patients had SBP (p=0.031) in Group B at follow up. During the period of follow up, only 1(4%) patient in Group A and 5(18%) patients in Group B died. There was no significant difference of number of death between two groups (p=0.196). Adrenal insufficient decompensated cirrhotic patients have higher morbidities.
 ...
PMID:Impact of Adrenocortical Insufficiency on Clinical Parameters in Haemodynamically Stable Cirrhotic Patients with Ascites. 2891 7