- T-Cell Large Granular Lymphocytic Leukemia - Case Reports. [Case Reports]
- ACActa Clin Croat 2018; 57(2):362-365
- T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on th...
T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain. Blood examination revealed mild mycrocitic anemia and multiplied lactate dehydrogenase level. Abdominal ultrasound showed splenomegaly of 16 cm, with no lymphadenopathy. Fine needle aspiration of bone marrow revealed hypocellular marrow with 50% of atypical lymphoid cells. There were 81% of atypical medium sized granular lymphocytes with irregularly shaped nuclei in peripheral blood, so the cytologic diagnosis was lymphoproliferative process. Bone marrow biopsy showed nodular and interstitial proliferation of small, partially atypical T lymphocytic cells positive for CD2, CD3, CD5, CD8, granzyme and TIA, and negative for hairy cell markers, CD10, MUM 1, bcl 1, CD4 and CD56. The finding was consistent with T-LGLL. Due to splenomegaly, the patient was treated with cyclosporine and gradually reduced dose of corticosteroids, leading to regression of splenomegaly and normalization of lactate dehydrogenase level.
- MLL-PTD in a 13-year-old patient with blast phase myeloproliferative neoplasm: A case report. [Journal Article]
- MMedicine (Baltimore) 2018; 97(46):e13220
- CONCLUSIONS: MLL-PTD occurs in the progression of JAK2V617F positive MPN into JAK2V617F negative AML, which may be a novel mechanism of MPN blast phase and helpful for post-MPN AML diagnosis. Allo-SCT may be a good choice for post-MPN AML with MLL-PTD. More therapeutic strategies need to be explored for a better prognosis in these patients.
- Successful resolution of Hemophagocytic lymphohistiocytosis associated to brucellosis in the adult. [Journal Article]
- TMTunis Med 2018; 96(7):458-461
- Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly di...
Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly diagnosed in the context of infections, malignancies and connective tissue diseases. Although brucellosis is an endemic infection in Tunisia, its association with HLH is a very rare condition which should be considered in patients with splenomegaly and cytopenia. Here, we describe brucellosis associated HLH in a 31 year-old man. The patient was admitted to our hospital with fever, sweating, and fatigue. Physical and laboratory findings revealed splenomegaly, pancytopenia, elevated serum transaminases, triglycerides, lactate dehydrogenase, and ferritin, and bone marrow hemophagocytosis. The Brucella agglutination test was positive. The patient improved after treatment with Rifampin and doxycyclin.
- Systemic lupus erythematosus complicated by noncirrhotic portal hypertension: A case report and review of literature. [Journal Article]
- WJWorld J Clin Cases 2018 Nov 06; 6(13):688-693
- A 48 year-old Chinese woman suffering from polyarthritis, irregular fever and trichomadesis was admitted to the hospital. A diagnosis of systemic lupus erythematosus (SLE) was made based on polyarthr...
A 48 year-old Chinese woman suffering from polyarthritis, irregular fever and trichomadesis was admitted to the hospital. A diagnosis of systemic lupus erythematosus (SLE) was made based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb's test and protein in her urine. In addition, splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging, indicating that the patient had SLE and portal hypertension. Further negative investigations ruled out the possibility of cirrhosis. The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension (NCPH) without liver histopathology, due to the patient's refusal for liver biopsy. Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone, hydroxychloroquine and metoprolol tartrate. To date, SLE complicated by NCPH has rarely been reported, as it is under-recognized clinically as well as pathologically. Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics, which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.
- Contrast-Enhanced Sonography in Patients with Hyposplenia: A Retrospective Analysis in Forty-Three Patients. [Journal Article]
- DDigestion 2018 Nov 13; :1-6
- CONCLUSIONS: Hyposplenia is a rare pathologic finding and often associated with hematological/oncological and autoimmune diseases. Furthermore, altered B-mode US appearance and a pathological CEUS pattern are frequently found. However, the clinical implication, especially regarding splenic function remains obscure to date.
- Acute Psychosis and Wilson's Disease. [Journal Article]
- QJMQJM 2018 Nov 13
- Wilson's Disease is an inborn error of metabolism associated with accumulation of copper in the body manifesting as hepatic, neurological and behavioural symptoms. Almost 20% patients initially prese...
Wilson's Disease is an inborn error of metabolism associated with accumulation of copper in the body manifesting as hepatic, neurological and behavioural symptoms. Almost 20% patients initially present with behavioural symptoms such as depression, personality changes or even mania. Schizophrenia and catatonia are less common. We describe the case of a 35 year old female with behavioural abnormalities diagnosed as a case of Schizophrenia and started on antipsychotics, which led to further worsening of her primary complaints. Physical examination was significant for ascites, splenomegaly and a right sided cataract. Hematological evaluation revealed pancytopenia. With a suspicion of underlying chronic liver disease associated with metabolic encephalopathy, she was evaluated for Wilson's disease, which was confirmed by biochemical and radiological evidence. Chelation therapy was initiated which led to complete reversal of her behavioural changes.
- Alpha1-Antitrypsin Deficiency: Transition of Care for the Child With AAT Deficiency into Adulthood. [Journal Article]
- CPCurr Pediatr Rev 2018 Nov 12
- CONCLUSIONS: AND RELEVANCE There should be a greater vigilance for AAT deficiency testing among pediatricians. Diagnosis should prompt assessment of liver involvement. Children with AAT-deficiency-associated liver disease should be referred to a liver specialist and monitored throughout their lifetimes for the symptoms of AAT-deficiency-related pulmonary involvement.
- A Review of Outcomes in Pregnant Women with Portal Hypertension. [Journal Article]
- JOJ Obstet Gynaecol India 2018; 68(6):447-451
- CONCLUSIONS: A multidisciplinary approach is essential to improve perinatal outcomes in pregnancy complicated by portal hypertension. Surgical measures to reduce portal venous pressure done before pregnancy or beta blockers during pregnancy might help reduce sudden variceal bleeds.
- Presenting signs and patient co-variables in Gaucher disease: outcome of the Gaucher Early Diagnosis Consensus (GED-C) Delphi initiative. [Journal Article]
- IMIntern Med J 2018 Nov 10
- CONCLUSIONS: The signs and co-variables identified in the GED-C initiative as potentially indicative of early GD will help to guide non-specialists and raise their index of suspicion in identifying patients potentially suitable for diagnostic testing for GD. This article is protected by copyright. All rights reserved.
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- Comparison of FIB-4 and transient elastography in evaluating liver fibrosis of chronic hepatitis C subjects in community. [Journal Article]
- PlosPLoS One 2018; 13(11):e0206947
- CONCLUSIONS: FIB-4 and TE defined different distribution of fibrosis stages in same HCV population. FIB-4 was deeply influenced by age whereas TE was not. TE had the advantages over than FIB-4 in strong association with splenomegaly and in detecting the role of non-alcoholic fatty liver disease in advanced fibrosis.