Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.
Humans ; Primary Myelofibrosis/drug therapy* ; Hypertension, Portal/complications* ; Liver Cirrhosis/pathology* ; Splenomegaly/pathology* ; Anemia

PURPOSE: To assess the relationship between splenic transient inhomogeneous contrast enhancement(CE) on the arterial phase of spiral CT, and splenic volume, and to classify the CE pattern in liver cirrhosis. MATERIALS AND METHODS: We measured the splenic volume of 120 patients, 60 showed inhomogeneous splenic CE on arterial phase,and 60 showed homogeneous splenic CE. CT scans with intrinsic splenic pathology were excluded. Sixteen patients with clinically confirmed liver cirrhosis were included. Splenic volumes of the inhomogeneous and homogeneous CE group were compared. The inhomogeneous group was divided into three grades according to areas of non-enhanced portion (grade I, focal geographic ; grade II, multifocal patchy, grade III, extensive serpentine inhomogeneous CE) , and these were correlated with splenic volume. RESULTS: Among the 60 inhomogeneous CE scans, 23 cases(38.3%) showed splenomegaly (spleen volume>220cm3); in contrast, this applied to only 8 cases (13.3%) of the 60 homogeneous CE scans. Mean splenic volume in the inhomogeneous CE group (226.74+/-129.78cm3) was greater than in the homogeneous CE group (184.56+/-77.44cm3) (p<0.033). A larger splenic volume and extensive inhomogeneous CE(grade III) were noted, and most liver cirrhosis patients(14/16) were grade III. Three such patients who had shown inhomogeneous splenic CE on arterial phase showed inhomogeneous CE even on portal phase. CONCLUSION: Inhomogeneous splenic CE on arterial phase was more common in cases of an enlarged spleen, and more extensive in liver cirrhosis. These findings suggest hemodynamic change of the spleen may be a contributory factor.
Hemodynamics ; Humans ; Liver Cirrhosis ; Pathology ; Spleen* ; Splenomegaly ; Tomography, Spiral Computed* ; Tomography, X-Ray Computed

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*

A case of histiocytic medullary reticulosis with splenic infaraction from a 23-year-old male is presented. Radiologic findings on selective spleen scintigraphy and abdominal CT are described. Selective spleen scintigraphy showed huge, multilobulated spleen with numerous photon-deficient areas in it and peripherally. Abdominal CT showed large peripheral band-like low density and infiltrative lesion in spleen with accompanying intraabdominal lymphadenopathy. Histoligical features were consistent with HMR in spleen and liver specimens.
Adult ; Hemoperitoneum/etiology ; Hepatomegaly/etiology/pathology ; Histiocytic Sarcoma/*complications ; Humans ; Male ; Splenic Infarction/*etiology/radiography/radionuclide imaging ; Splenic Rupture/etiology ; Splenomegaly/etiology/pathology ; Tomography, X-Ray Computed

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. After many years, a few patients with ET may develop bone marrow (BM) fibrosis and rarely develop osteosclerosis. A 60-yr-old female was admitted due to severe left upper quadrant abdominal discomfort. She had been diagnosed as ET 19 yrs ago. On liver computed tomography severe splenomegaly was shown. Laboratory tests revealed WBC 24.3x10(9)/L, hemoglobin 13.4 g/dL, platelets 432x10(9)/L, lactate dehydrogenase 4,065 IU/L (reference range; 240-480). Blood smear demonstrated leukoerythroblastosis, teardrop cells, and giant and hypogranular platelets. BM study revealed inadequate aspirate due to dry tap. BM biopsy showed clusters of dysplastic megakaryocytes, grade 3 fibrosis, and severe osteosclerosis. Major/minor BCR-ABL1 rearrangement and JAK2 V617F mutation were not detected. Cytogenetic studies revealed normal karyotype. According to the 2008 WHO diagnostic criteria, the patient was diagnosed as having post-essential thrombocythemia myelofibrosis with severe osteosclerosis.
Bone Marrow/pathology ; Female ; Humans ; Megakaryocytes/pathology ; Middle Aged ; Osteosclerosis/complications/*diagnosis ; Primary Myelofibrosis/complications/*diagnosis ; Splenomegaly/etiology ; Thrombocythemia, Essential/complications/*diagnosis ; Tomography, X-Ray Computed

Adult ; Arsenic Poisoning ; pathology ; Chronic Disease ; Hemosiderin ; metabolism ; Hemosiderosis ; metabolism ; pathology ; Humans ; Hypertension, Portal ; chemically induced ; metabolism ; pathology ; Liver Cirrhosis ; chemically induced ; metabolism ; pathology ; Male ; Pancytopenia ; chemically induced ; metabolism ; pathology ; Splenomegaly ; chemically induced ; metabolism ; pathology

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.
Biopsy* ; Bone Marrow ; Brain Diseases ; Brain* ; Central Nervous System ; Cerebrospinal Fluid ; Diagnosis ; Fever ; Humans ; Jaundice ; Leukocytosis ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pathology ; Splenomegaly

OBJECTIVETo understand the clinical feature and natural course of polycythemia vera (PV).

METHODSThe clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed.

RESULTSThere are 122 males and 63 females. The mean age was (52.7 +/- 14.1) years. The mean hemoglobin level was (208.3 +/- 21.2) g/L. Pancytosis was displayed in 74 (40%) cases, excess of red blood cells in 33 (17.8%), excess of red blood cells and granulocytes in 67 (36.2%) and excess of red blood cell and platelets in 11 (5.9%). Splenomegaly was found in 123 (66.5%) patients and hepatomegaly in 30 (16.2%). Quantitative assess of serum Epo was done in 25 patients. The level was low in 16 (64.2%) and normal in 9 (36.0%). Hematopoietic progenitor culture yields was elevated in 11 patients, endogenous erythroid colonies (EEC) formation was found in 10 cases (90.9%). Eighty two patients (44.3%) had 101 attacks of vascular thrombotic incidents, 7 patients developed myelofibrosis (MF). Secondary cancer occurred in 1 patient. Two patients died of thrombosis.

CONCLUSIONPV is an elderly adult myeloproliferative disease with a high frequency of thrombosis. EEC can be found out in PV patients. The serum Epo level is not increased in PV patients. The main sequelae of PV is MF.

Adult ; Aged ; Erythrocyte Count ; Female ; Hemoglobins ; metabolism ; Hepatomegaly ; etiology ; Humans ; Leukocyte Count ; Male ; Middle Aged ; Polycythemia Vera ; blood ; complications ; pathology ; Primary Myelofibrosis ; etiology ; Splenomegaly ; etiology ; Thrombosis ; etiology

Adult ; Aged ; Esophageal and Gastric Varices ; diagnosis ; surgery ; Female ; Humans ; Male ; Middle Aged ; Pancreas ; pathology ; surgery ; Pancreatic Neoplasms ; diagnosis ; surgery ; Splenomegaly ; diagnosis ; surgery ; Young Adult