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

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

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*

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

OBJECTIVETo assess the feasibility and safety of radiofrequency ablation (RFA) in spleen to treat secondary splenomegaly and hypersplenism in dogs.

METHODSFourteen healthy mongrel dogs were randomly divided into two groups: group A (n = 4) and group B (n = 10) Both groups underwent ligation of the splenic vein and its collateral branches to induce congestive splenomegaly. At the end of the 3rd week, radiofrequency thermal ablation of the spleen was performed in the group B via laparotomy. After splenic RFA, the procedure-related complications were observed, CT scan was performed and the spleens were obtained according to schedule. The radiofrequency (RF) thermal lesions and its histo- pathological changes of the spleen were examined regularly.

RESULTSThere were no morbidity and mortality in the experimental dogs. CT findings revealed that splenomegaly could sustained over 2 months after ligation of the splenic vein. The segmental RF lesions included hyperintense zone of coagulative necrosis and more extensively peripheral hypo-intense infarcted zone. The latter was called as "bystander effect". The infarcted zone would be absorbed and subsequently disappeared between 4 and 6 weeks after RFA, and the size of the remnant spleen shrunk, but the lesion of coagulative necrosis hardly altered. The fundamental histopathological changes of splenic lesions caused by RF thermal energy included local coagulative necrosis and the peripheral thrombotic infarction zone. Subsequently, tissue absorption and fibrosis occurred in the zone of thrombotic infarction. Simultaneously occluded vessels, fibrin deposition, and disappearance of normal splenic sinuses resulted in the condensed structure of the viable remnant spleen, which were the pathological basis responsible for the shrunk spleen.

CONCLUSIONSIt is feasible and safe to perform RFA in the spleen to treat experimental splenomegaly and hypersplenism. The RFA technique could be safely performed clinically via laparotomy or laparoscopic procedure to strictly isolate the spleen from the surrounding organs.

Animals ; Catheter Ablation ; Disease Models, Animal ; Dogs ; Feasibility Studies ; Female ; Hypersplenism ; etiology ; pathology ; surgery ; Ligation ; adverse effects ; Male ; Random Allocation ; Spleen ; diagnostic imaging ; pathology ; Splenectomy ; methods ; Splenic Vein ; surgery ; Splenomegaly ; etiology ; pathology ; surgery ; Tomography, X-Ray Computed

OBJECTIVETo explore the clinical characteristics of Wolman disease and diagnostic methods using enzymatic and molecular analysis.

METHODLysosomal acid lipase activity was measured using 4-methylumbelliferyl oleate in the leukocytes of an infant suspected of Wolman disease and LIPA gene mutational analysis was performed by PCR and direct sequencing in the proband and his parents. After the diagnosis was confirmed, the clinical, biochemical, radiological and histopathological findings in this case of Wolman disease were retrospectively reviewed.

RESULTThe sixteen-day-old boy was failing to thrive with progressive vomiting, abdominal distention and hepatosplenomegaly. Abdominal X-ray revealed adrenal calcifications which were confirmed on abdominal CT scan. Xanthomatosis were observed on enlarged liver, spleen and lymph nodes during abdominal surgery. Liver and lymph node biopsy showed foamy histiocytes. The lysosomal acid lipase activity in leukocytes was 3.5 nmol/(mg·h) [control 35.5 - 105.8 nmol/(mg·h)]. Serum chitotriosidase activity was 315.8 nmol/(ml·h) [control 0 - 53 nmol/(ml·h)]. The patient was homozygote for a novel insert mutation allele c.318 ins T, p. Phe106fsX4 in exon 4 on LIPA gene. His both parents were carriers of the mutation.

CONCLUSIONThe clinical features of Wolman disease include early onset of vomiting, abdominal distention, growth failure, hepatosplenomegaly and bilateral adrenal calcification after birth. A plain abdominal X-ray film should be taken to check for the typical pattern of adrenal calcification in suspected cases of Wolman disease. The enzymatic and molecular analyses of lysosomal acid lipase can confirm the diagnosis of Wolman disease.

Adrenal Gland Diseases ; etiology ; pathology ; Exons ; Humans ; Infant, Newborn ; Leukocytes ; enzymology ; Lipase ; blood ; genetics ; Liver ; pathology ; Lysosomes ; enzymology ; genetics ; Male ; Mutation ; Polymerase Chain Reaction ; Splenomegaly ; pathology ; Sterol Esterase ; genetics ; Tomography, X-Ray Computed ; Wolman Disease ; diagnosis ; enzymology ; genetics ; pathology

Aloe is one of the leading products used in phytomedicine. Several cases of aloe-induced toxic hepatitis have been reported in recent years. However, its toxicology has not yet been systematically described in the literature. A 21-year-old female patient was admitted to our hospital with acute hepatitis after taking an aloe vera preparation for four weeks. Her history, clinical manifestation, laboratory findings, and histological findings all led to the diagnosis of aloe vera-induced toxic hepatitis. We report herein on a case of acute toxic hepatitis induced by aloe vera.
Adult ; Aged ; Alanine Transaminase/blood ; Alkaline Phosphatase/blood ; Aloe/*chemistry/metabolism ; Drug-Induced Liver Injury/*diagnosis/etiology/pathology ; Female ; Humans ; Liver/pathology ; Male ; Middle Aged ; Plant Extracts/*adverse effects/*chemistry ; Splenomegaly/diagnosis ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo study the effect of spleen lymphocytes on the splenomegaly by hepatocellular carcinoma-bearing mouse model.

METHODSCell counts, cell cycle distribution, the percentage of lymphocytes subsets and the levels of IL-2 were measured, and two-dimensional gel electrophoresis (2-DE) was used to investigate the relationship between spleen lymphocytes and splenomegaly in hepatocellular carcinoma-bearing mice.

RESULTSCompared with the normal group, the thymus was obviously atrophied and the spleen was significantly enlarged in the tumor-bearing group. Correlation study showed that the number of whole spleen cells was positively correlated with the splenic index. The cell diameter and cell-cycle phase distribution of splenocytes in the tumor-bearing group showed no significant difference compared to the normal group. The percentage of CD3+ T lymphocytes and CD8+ T lymphocytes in spleen and peripheral blood of tumor-bearing mice were substantially higher than that in the normal mice. Meanwhile, the IL-2 level was also higher in the tumor-bearing group than in the normal group. Furthermore, two dysregulated protein, β-actin and S100-A9 were identified in spleen lymphocytes from H22-bearing mice, which were closely related to cellular motility.

CONCLUSIONIt is suggested that dysregulated β-actin and S100-A9 can result in recirculating T lymphocytes trapped in the spleen, which may explain the underlying cause of splenomegaly in H22-bearing mice.

Animals ; Carcinoma, Hepatocellular ; complications ; Cell Cycle ; Female ; Liver Neoplasms ; complications ; Lymphocytes ; physiology ; Mice ; Mice, Inbred ICR ; Neoplasms, Experimental ; therapy ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; Spleen ; cytology ; pathology ; Splenomegaly ; etiology ; therapy ; Thymus Gland

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
Alanine Transaminase/metabolism ; Alkaline Phosphatase/metabolism ; Antigens, CD/blood ; Antigens, Differentiation, Myelomonocytic/blood ; Arthritis, Juvenile Rheumatoid/blood/*complications/pathology ; Aspartate Aminotransferases/metabolism ; Blood Cell Count ; Hepatomegaly/*etiology/pathology ; Humans ; Infant ; Liver/enzymology/pathology ; *Macrophage Activation ; Male ; Partial Thromboplastin Time ; Prothrombin Time ; Splenomegaly/*etiology/pathology ; Syndrome ; gamma-Glutamyltransferase/metabolism

Previous studies reported that oxaliplatin is associated with sinusoidal obstruction syndrome. However few reports on oxaliplatin induced liver fibrosis are found in the literature. Furthermore pathogenesis of liver fibrosis is not well known. We report a case of 45-yr-old Korean man in whom liver fibrosis with splenomegaly developed after 12 cycles of oxaliplatin based adjuvant chemotherapy for colon cancer (T4N2M0). Thorough history taking and serological examination revealed no evidence of chronic liver disease. Restaging CT scans demonstrated a good response to chemotherapy. Five month after chemotherapy, he underwent right hepatectomy due to isolated metastatic lesion. The liver parenchyma showed diffuse sinusoidal dilatation and centrilobular vein fibrosis with necrosis without steatosis. We could conclude that splenomegaly was due to perisinusoidal liver fibrosis and liver cell necrosis induced portal hypertension by oxaliplatin. In addition, to investigate the pathogenesis of liver fibrosis, immunohistochemical stains such as CD31 and alpha-smooth muscle actin (alpha-SMA) were conducted with control group. The immunohistochemical stains for CD31 and alpha-SMA were positive along the sinusoidal space in the patient, while negative in the control group. Chemotherapy with oxaliplatin induces liver fibrosis which should be kept in mind as a serious complication.
Actins/metabolism ; Antigens, CD31/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Camptothecin/*analogs & derivatives/therapeutic use ; Chemotherapy, Adjuvant ; Colonic Neoplasms/*drug therapy ; Fluorouracil/therapeutic use ; Humans ; Hypertension, Portal/etiology ; Immunohistochemistry ; Leucovorin/therapeutic use ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Liver Neoplasms/secondary/surgery ; Male ; Middle Aged ; Organoplatinum Compounds/*administration & dosage/adverse effects/therapeutic use ; Splenomegaly/*diagnosis/etiology ; Thrombocytopenia/etiology ; Tomography, X-Ray Computed