Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Burkitt's lymphoma (BL) is a major cause of death among Ugandan children. We studied clinical characteristics and outcomes of childhood BL over time at the Uganda Cancer Institute (UCI). A total of 1217 children (766 boys, 451 girls, mean age 6.69 years) diagnosed with BL between 1985 and 2005 were included. There were no significant changes in the proportion of boys and girls diagnosed, or in mean age at diagnosis. Facial tumor (n=945, 77.65%) and abdominal disease (n=842, 69.19%) were the most common presentations. The proportion of children presenting with hepatic mass, malignant pleocytosis, and advanced-stage (stage C and D) BL increased during the study period (P<0.01). A total of 1085 children out of 1206 (89.97%) received at least one cycle of chemotherapy, and 832 of 1099 (75.71%) demonstrated objective response (i.e. complete or partial remission). The most common symptoms at BL diagnosis were fever (n=621, 51.03%), anemia (n=593, 48.73%), and weight loss (n=588, 48.32%). Significant increases in the proportion of children with fever, and significant changes in the proportion of children with anemia, night sweats and severe infection were observed. HIV positivity was 3.87%, but no substantial differences in the proportion of HIV-positive children were observed. Mortality was not significantly different over time: it was similar in boys and girls, higher in older children (compared with younger ones), in those with advanced-stage BL, and HIV-positive children, but lower in children with facial tumors compared with other tumor presentations, and among those who received chemotherapy.
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PMID:Clinical characteristics, treatment and outcome of childhood Burkitt's lymphoma at the Uganda Cancer Institute. 2204 12

A cross-sectional study on pulmonary TB diagnosis delay in an intermediate TB incidence setting showed average patient's delay of 44 +/- 61.65 days and total delay of 103 +/- 148 days. Alcoholism, lack of TB cases in family, diabetes mellitus, relapse, cough or tachycardia (p< 0.01), absence of hemoptysis, dyspnea and anemia (p < 0.01), age > or = 40 (p < 0.05), negative auscultation and positive sputum smear findings (p < 0.05) were significantly associated with patient's delay > 30 days. Age < 40 years, negative auscultation and sputum smear findings (p < 0.01), female sex, city as residence (p < 0.05), absence of cough, sputum, weight loss, fever, excavation (p < 0.01), and night sweats (p < 0.05) were significantly associated with total delay > 103 days. Further population education and continual medical education are waranted.
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PMID:Is there delay in diagnosis of pulmonary tuberculosis in an intermediate-to-low TB incidence setting. 2209 35

Little is known about referrals from primary care providers (PCPs) for suspected hematologic malignancies, including their clinical triggers and frequency. A random sample of 190 Massachusetts PCPs were presented with a vignette concerning a patient with a new finding of moderate anemia, asked how they would respond, and then asked what they would do if the patient returned with persistent anemia plus one additional sign or symptom. We also asked about referral behaviors for suspected hematologic malignancies during the prior year. A total of 134 (70.5%) PCPs responded. At first anemia presentation,only 3.8% reported referring to hematology. The development of a second sign or symptom yielded higher referral rates: pancytopenia 588.7%, leukopenia 5 63.9%, thrombocytopenia 5 63.9%, lymphadenopathy 5 42.9%, leukocytosis 5 37.6%, night sweats 5 25.6%, and weight loss 5 23.3%. The median yearly number (interquartile range) of patients PCPs reported suspecting of having hematologic malignancy was 5 (3, 10), and the median formally referred was 5 (3, 10). We conclude that anemia plus signs and symptoms suggestive of myelodysplasia or leukemia (compared with those suggestive of lymphoma) are more likely to prompt hematology referral. In addition, given their rarity,the numbe
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PMID:Referrals for suspected hematologic malignancy: a survey of primary care physicians. 2247 54

Tuberculosis (TB) is still one of the most frequent infectious diseases worldwide. Until the 1990s, Western European countries showed a low frequency of TB infection, but the rise of immigration has led to a rapid increase in its occurrence. In the elderly, TB is emerging as a significant health problem (age-related decline of the cell-mediated immunity, associated illnesses, use of immunosuppressive drugs, malnutrition, poor life conditions), although its detection and diagnosis is not easy also considering its subclinical presentation. Almost 70% of all TB infections in Italy are found in the lungs; 50% of the extrapulmonary infections affect lymph nodes. Due to the low incidence of superficial tuberculous lymphadenitis without pulmonary manifestations, the possibility of a TB aetiology is often not taken into consideration in the differential diagnosis of lymphadenopathy, resulting in significant delay of appropriate treatment. Herein, we describe the case of a 78-year-old male with nocturnal fever, weakness, night sweats, loss of weight and decay in general condition. The patient had a past medical history of prostate adenocarcinoma treated with hormone therapy. The past medical history in association with clinical findings and laboratory data (anaemia, high titers of fibrinogen and reactive C-protein) led to the suspect of metastatic adenocarcinoma. Only histological and molecular biology findings allowed us to make a correct diagnosis of TB.
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PMID:Tuberculosis of superficial lymph nodes, a not so rare event to consider in diagnosis. A case in an elderly male. 2255 92

Castleman and Towne described a disease presenting as a mediastinal mass resembling thymoma. It is also known as "giant lymph node hyperplasia", "lymph node hamartoma", "angiofollicular mediastinal lymph node hyperplasia", and "angiomatous lymphoid hyperplasia". The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue. In addition to the mediastinal presentation, extrathoracic involvement in the neck, axilla, mesentery, pelvis, pancreas, adrenal gland, and retroperitoneum also have been described. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular variant, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell variant, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergamma-globulinemia. Castleman disease is a rare lymphoproliferative disorders. Few cases have been described world widely. In this article we reviewed the classification, pathogenesis, pathology, radiological features and up to date treatment with special emphasis on the role of viral stimulation, recent therapeutic modalities and the HIV-associated disease.
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PMID:Castleman disease. 2307 71

Myelofibrosis (MF) is characterized by splenomegaly, anemia and a debilitating symptom burden (e.g., fatigue, night sweats, pruritus, bone and muscle pain, undesired weight loss). Moreover, these symptoms impair activities of daily living and quality of life. Until recently, there have been no approved therapies for MF, and management of MF has been predominantly palliative. Dysregulated JAK-STAT signaling is associated with the pathologic MF disease state. A novel class of therapies, the JAK inhibitors, offers the potential to abrogate this pathologic signaling pathway. In clinical trials of patients with intermediate- and high-risk MF, JAK inhibitors have demonstrated efficacy in reducing splenomegaly and MF-associated symptoms. Evidence from ruxolitinib trials also suggests that JAK inhibitors may improve survival outcomes.
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PMID:Assessing efficacy in myelofibrosis treatment: a focus on JAK inhibition. 2321 93

A 62-year-old man with no significant medical history experienced fatigue, night sweats, hoarseness of voice, and dry cough, which were followed by vision disturbances in his left eye. He lost about 4.5 kg (10 lb) in just over a month. Three weeks later, he had difficulty recollecting his e-mail password and trouble with word finding. The next day, he experienced numbness in his left arm. He also developed a maculopapular and erythematous rash in the groin, genitalia, and buttocks. The results of an initial neurological examination were normal, including his higher mental functions. An initial blood workup revealed normocytic normochromic anemia. The results of cerebrospinal fluid studies were unremarkable. Magnetic resonance imaging of his brain at hospital admission revealed multifocal increased T2 signals in the subcortical white matter. A conventional cerebral angiogram was unremarkable. A biopsy specimen from the right frontal lobe revealed demyelination and perivascular lymphocytic infiltration. A provisional diagnosis of acute disseminated encephalomyelitis was made. In spite of steroid treatment and plasmapheresis, his clinical status deteriorated rapidly. The approach to the diagnosis of a rapidly progressive multifocal brain disorder is discussed.
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PMID:A 62-year-old man with fluctuating neurological deficits and skin lesions. 2383 17

Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are referred to as the classic Philadelphia chromosome (BCR-ABL1)-negative myeloproliferative neoplasms. Although each has distinct pathologic features, all 3 display alterations in Janus kinase (JAK) signal transduction activator of transcription signaling. Myelofibrosis is the most serious of the 3, associated with shortened survival (median survival, 5-7 years); bone marrow failure with anemia; progressive splenomegaly; and chronic, burdensome symptoms, including fatigue, night sweats, itching, abdominal discomfort, loss of appetite/early satiety, unintentional weight loss, and bone, chest, and abdominal pain. Treatments for MF have been mainly palliative, with the exception of allogeneic stem cell transplantation, which, although potentially curative, is feasible only in a small subpopulation of patients. In November 2011, ruxolitinib, an inhibitor of JAK1 and JAK2, was approved by the US Food and Drug Administration for the treatment of intermediate- or high-risk MF, including primary MF, post-PV MF, and post-ET MF. In clinical trials, ruxolitinib was shown to reduce spleen volume and improve MF-related symptoms and quality-of-life measures. Evidence also suggests that ruxolitinib therapy has a survival advantage over placebo and best available therapy. Thrombocytopenia and anemia were the most common adverse events with treatment. Ongoing trials are assessing the efficacy and safety of ruxolitinib therapy in patients with PV and ET.
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PMID:Ruxolitinib: an oral Janus kinase 1 and Janus kinase 2 inhibitor in the management of myelofibrosis. 2339 78

Castlemans disease (also called angiofollicular lymph node hyperplasia) can take two forms with different prognosis: the localized form can usually be treated by a surgical intervention and has therefore a favourable prognosis. On the other hand, the multicentric form has an unfavourable prognosis and requires systemic treatment. Classic manifestations of multicentric Castlemans disease are multiple sites of lymphadenopathy, sometimes hepatomegaly and also splenomegaly or serous cavity effusions. Typical pathological laboratory levels measured in patients with this disease include an increased CRP level, anaemia of chronic diseases, and many patients have an increased total protein concentration, in some cases exceeding even 100g/ l. It is caused by a high concentration of polyclonal immunoglobulins. Typical clinical symptoms include fluctuating subfebrile or febrile temperatures, increased night sweats and fatigue usually related to anaemia. In some patients, the disease is manifested as vasculitis, frequently also affecting cerebral arteries, i.e. leading to cerebrovascular accidents. The aetiology of this disease is unclear; it is a polyclonal lymphocyte proliferation, often with differentiation into plasma cells. It is not a clonal malign disease; however, it can transform into a clonal lymphoproliferative disease. Even though it is not a malign disease in the histomorphological sense, the disease symptoms are so acute that systemic treatment is required. In the past, the treatment method of this disease used to be based on corticoids and cytostatics; however, such treatment was not always successful in achieving its objective, i.e. complete remission. In the past few years, an improvement of treatment results was accomplished by adding a new drug to the basic medication, i.e. to cytostatics and dexamethasone. Many publications describe the benefi t of adding a third drug from the IMiDs group (immunomodulatory drugs), such as thalidomide or lenalidomide. These drugs affect the formation of cytokines and block the angiogenesis, which in turn positively influences the speed of the treatment response. The second new drug that has helped in combination with classical treatment is the anti-CD20 antibody, rituximab. The third new drug to add this list is the monoclonal antibody against the interleukin-6 receptor, tocilizumab. This paper describes a rapid treatment response after combined treatment with cyclophosphamide 500mg/ m2 i.v. infusion 1st and 15th day in a 28- day cycle, dexamethasone 20mg p.o. cycle day 1- 4 and cycle day 15- 18, and thalidomide 100mg daily. In the course of the two-month treatment, the accumulation of fl uorodeoxyglucose during the PET-CT imaging has normalized; the originally pathologically enlarged nodes have become smaller, the originally elevated CRP level has normalized and the originally signifi cantly lower haemoglobin level has risen. This is the second patient with multicentric Castlemans disease in the last three years who showed a rapid response to treatment with thalidomide combined with cyclophosphamide and dexamethasone. Therefore, we consider such treatment suitable for newly diagnosed patients with multicentric Castlemans disease.
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PMID:[PET-CT documented fast onset of treatment response to cyclophosphamide, thalidomide and dexamethasone in patients with multicentric Castlemans disease. Case description and treatment information overview]. 2371 Oct 57

Initial identification of chronic myelogenous leukemia is very important since targeted therapy leads to life-saving remission. Rarely, chronic myelogenous leukemia presents with an unusual picture, making the diagnosis challenging. We describe such a case of chronic myelogenous leukemia in blast crisis in a previously healthy 61-year-old woman. The patient presented with fever, myalgias, and night sweats and was first worked up for an infectious etiology. Because of persistent anemia, a bone marrow biopsy was performed that revealed fibrosis with increased megakaryoblasts. Even though initial cytogenetic studies could not be performed because of "dry tap" aspirate, persistent efforts for cytogenetic studies were made, including a "squeeze preparation" from the core biopsy, which revealed t(9;22)(q34;q11.2) and trisomy 19. The patient was treated with tyrosine kinase inhibitors, chemotherapy, and subsequently an allogeneic stem cell transplant. She is in persistent remission. This case illustrates a complex presentation of chronic myelogenous leukemia and provides an overview of morphologic cues and the importance of performing cytogenetic studies that led to the diagnosis.
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PMID:An unusual case of Philadelphia chromosome-positive chronic myelogenous leukemia with trisomy 19 presenting with megakaryoblastosis and myelofibrosis. 2389 72


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