Fever ; Humans ; Oliguria ; diagnosis ; Recurrence ; Splenomegaly ; diagnosis

Cystic lymphangioma of the spleen is extremely rare disease and the treatment is always surgical. In the present report we describe a symptomatic splenic cystic lymphangioma in a 44 year-old female. Clinical examination revealed palpable mass in the left upper quadrant of the abdomen. Abdominal CT scan revealed enlarged spleen with multiple, variable sized cysts. A few cysts had peripheral calcification. Open splenectomy was performed and histologic examination permitted the accurate diagnosis to be made. In case of multiple cystic enlargement of spleen, consider the possibility of cystic lymphangioma of the spleen and splenectomy.
Abdomen ; Adult ; Diagnosis ; Female ; Humans ; Lymphangioma, Cystic* ; Rare Diseases ; Spleen* ; Splenectomy ; Splenomegaly ; Tomography, X-Ray Computed

Anemia ; complications ; Child ; Female ; Fever ; complications ; Humans ; Hypereosinophilic Syndrome ; complications ; diagnosis ; therapy ; Splenomegaly ; complications

Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder characterized by cytopenia, splenomegaly and mononuclear cells displaying cytoplasmic projections. Diagnosis is based on the distinctive hairy cell morphology and immunological profile. In the last 10 to 15 years the prognosis of patients with HCL has improved considerably following the use of purine analogues such as deoxycoformycin and 2-chlorodeoxyadenosine (2-CdA). We report 3 patients with HCL who were treated with 2-CdA at a daily dosage of 0.1mg/kg by continuous intravenous infusion for 7 days. After 1 or 2 courses of treatment, all patients achieved complete remission and are still alive in disease-free status.
B-Lymphocytes ; Cladribine* ; Cytoplasm ; Diagnosis ; Humans ; Infusions, Intravenous ; Leukemia, Hairy Cell* ; Lymphoproliferative Disorders ; Pentostatin ; Prognosis ; Splenomegaly

OBJECTIVETo compare the clinical features of children with different clinical forms of congenital hepatic fibrosis (CHF), and provides a description of the characteristics of childhood CHF.

METHODSSixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF. The medical data of 26 children with PH CHF and 30 children with mixed CHF, including gender, age, clinical manifestations, physical signs, laboratory tests and imaging characteristics, were retrospectively studied.

RESULTSFever, jaundice and hepatomegaly were more frequently noted in children with mixed CHF than in those with PH CHF (P<0.05). Splenomegaly and liver cirrhosis occurred more often in children with CHF, but there was no significant difference in the incidences of splenomegaly and liver cirrhosis between the children with PH CHF and mixed CHF. The plasma prothrombin activity, white blood cell counts, platelet counts, mean platelet volume, serum levels of alanine transaminase, aspartate transaminase, alkaline phosphatase, γ-glutamyl transferase, leucine aminopeptidase, and total bile acids in children with mixed CHF were higher than in those with PH CHF (P<0.05). The decreased international normalized ratio and lower serum albumin levels were more frequently observed in children with mixed CHF than in those with PH CHF (P<0.05).

CONCLUSIONSPH and mixed CHF are common forms in childhood CHF. The children with the two forms of PH usually manifest portal hypertension such as cirrhosis and hepatosplenomegaly. The liver damage may be common in children with mixed CHF.

Adolescent ; Alkaline Phosphatase ; blood ; Child ; Female ; Genetic Diseases, Inborn ; complications ; diagnosis ; Humans ; Liver Cirrhosis ; complications ; diagnosis ; Male ; Splenomegaly ; etiology

OBJECTIVETo present six cases of Brucella infection in children, analyze the characteristics of the disease, diagnostic and therapeutic process.

METHODThe clinical manifestations, laboratory test results and diagnostic process of 6 confirmed cases of brucellosis seen between 2011-2012 were retrospectively analyzed and domestic and foreign literature was reviewed.

RESULTAll the 6 children had a history of either exposure to, travelling to endemic area, or consuming infected lamb/beef. After the relevant examinations for these children, either positive etiologic or serologic evidence of brucellosis infection was obtained. The main clinical manifestation was fever in all cases, the peak body temperature was 37.5-38.0 °C in 3 cases, 38.1-39.0 °C in 2 cases, 39.1-41 °C in 1 case. Except for 1 case whose fever type was undulant fever, all the rest had irregular fever.Joint pain existed in 3 cases, orchitis in 1 case, cervical lymphadenopathy in 3 cases, hepatosplenomegaly in 2 cases, and impaired liver function in 4 cases. The Brucella agglutination test was positive in 5 cases. The blood culture was positive for all cases. In 4 cases the sulfamethoxazle and rifampicin were used for treatment, 1 case was treated with rifampicin and erythromycin, parents of 1 case refused to use the drug. The "brucellosis in children" was used to search literature at Wanfang database, Pubmed database for literature of recent 10 years, and a total of 13 articles including 15 cases were retrieved. All the patients had fever, 6 cases had joint swelling and pain, 10 cases had hepatosplenomegaly, 6 cases had cervical lymphadenopathy, 4 cases were complicated with central nervous system infection. Brucella agglutination test was positive in 9 cases and blood culture was positive for Brucella infection in all cases.

CONCLUSIONChildhood Brucella infections are usually presented with various clinical manifestations, and are often accompanied by symptoms of systemic infection. For fever of unknown origin, one should include tests associated with brucellosis and pay special attention to differential diagnosis against other diseases.

Animals ; Brucella ; Brucellosis ; diagnosis ; pathology ; Cattle ; Child ; Diagnosis, Differential ; Fever ; Humans ; Lymphatic Diseases ; pathology ; Meat ; Retrospective Studies ; Sheep ; Splenomegaly ; pathology

Hemophagocytic lymphohistiocytosis (HLH) was a life-threatening syndrome due to the uncontrolled immune activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages. HLH is characterized by primary and secondary causes, the early diagnosis and treatment of patients are closely related to the prognosis and clinical outcome of patients. The clinical presentation is variable but mostly includes prolonged fever, splenomegaly, coagulopathy, hypertriglyceridemia, and hemophagocytosis, none of them is specific and particular for HLH. Tuberculosis (TB) infection is one of the causes of HLH. HLH caused by TB is very rare clinically, but it has a high mortality. For patients with fever of unknown origin, HLH-related clinical manifestations sometimes present before the final diagnosis of TB, and HLH is associated with the most significant mortality rate. This article is mainly about a 28-year-old patient with HLH who suffered from severe TB infection. The patient attended a hospital with a history of 2 months of prolonged fever, 10 days booger and subcutaneous hemorrhage in lower limbs. Before this, he was in good health and denied any history of tuberculosis exposure. Combined with relevant laboratory test results (such as splenomegaly, hemoglobin, platelet count, and hypertriglyceridemia) and clinical manifestations (e.g. fever), the patient was diagnosed with hemophagocytic lymphohistiocytosis, but the etiology of HLH remained to be determined. To confirm the etiology, the patient was asked about the relevant medical history (intermittent low back pain) and was performed chest CT scan, bone marrow biopsy, and fundus photography. Finally, he was diagnosed with hemophagocytic lymphohistiocytosis caused by hematogenous disseminated pulmonary tuberculosis. In response to this, intravenous methylprednisolone and anti-tuberculosis treatment (isoniazid, pyrazinamide, moxifloxacin, and amikacin) were administered to the patient. After more than a month of treatment, the patient recovered from HLH caused by severe TB infection. Therefore, this case suggests that we should be vigilant to the patient who admitted to the hospital with fever for unknown reasons, to diagnose HLH as early as possible and clarify its cause, then perform interventions and treatment, especially HLH secondary to tuberculosis. Also, cases of atypical TB and severe TB should be carefully monitored to achieve early diagnosis and early intervention.
Male ; Humans ; Adult ; Lymphohistiocytosis, Hemophagocytic/diagnosis* ; Splenomegaly ; Tuberculosis, Pulmonary/diagnosis* ; Bone Marrow/pathology* ; Fever/etiology* ; Hypertriglyceridemia/complications*

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.
Female ; Hepatomegaly/diagnosis ; Humans ; Infant ; Langerhans Cell Sarcoma/*diagnosis ; Magnetic Resonance Imaging ; Mediastinal Neoplasms/*diagnosis ; Neoplasm Recurrence, Local ; Skull Neoplasms/*diagnosis ; Splenomegaly/diagnosis ; Tomography, X-Ray Computed

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.
Anemia ; Anemia, Hemolytic ; Bilirubin ; Child* ; Diagnosis ; Erythrocytes ; Gilbert Disease* ; Hemolysis ; Humans ; Hyperbilirubinemia ; Jaundice* ; Male ; Nausea ; Osmotic Fragility ; Spleen ; Splenomegaly ; Thalassemia

Sixty two properly treated acute lymphocytic leukemia cases who were diagnosed at SNUH, from 1958 to July, 1981 were studied. Among them, 20 cases had survived more than 3 years and 42 cases survived less than 3 years. The 11 cases of 20 are now alive and off theray and the remainder 9 cases were expired after survival for more than three years. The clinical features of the 11 currently living cases are as follows. The survival time of 11 cases are 3 years and 1 month - 16 years and 1 month. Except one case, first remission have continued till now. Average leukocyte count and liver size are 7,710/mm3 and 1.5 fingerbreadth respectively. The proportion of cases who showed splenomegaly, lymph node enlargement and hemorrhagic phenomena is 18.2%, 9.1% and 36.4% respectively. The clinical features of the 9 expired cases are as follows. Average leukocyte count is 7,780/mm3 and average liver size is 2.4 finger-breadth and average spleen size is 2.8 fingerbreadth. The proportion of cases who showed lymph node enlargement and hemorrhagic phenomean in 50.0% and 37.5% respectively. The clinical features of 29 cases who attained initial remission but survived less than 3 years are as follows. Average leukocyte count, liver size and spleen size 72,910/mm3, 2.8 finger-breadth and 2.0 finger-breadth respectively. The proportion of cases who showed lymph node enlargement and hemorrhagic phenomean is 52% and 75.9% respectively. The clinical features of 13 cases who died before initial remission are as follows. Average leukocyte count, liver size and spleen size are 244,570/mm3, 2.4 finger-breadth and 2.0 finger-breadth respectively. The proportion of cases who showed lymph node enlargement and hemorrhagic phenomena is 53.8% and 90.9% respectively. Various prognostic factors were related to duration of survival. The proportion of cases who were over 10 years of age at diagnosis and male to female ratio are higher in cases who survived less than 3 years. The leukocyte count, liver size, proportion of lymph node enlargement and proportion of hemorrhagic phenomeana are significantly lower in long survivors. In long survivors, spleen size in smaller but statistically not significant.
Diagnosis ; Female ; Humans ; Leukocyte Count ; Liver ; Lymph Nodes ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Spleen ; Splenomegaly ; Survivors