Correlation between the values of circulating blood elements with the size of spleen in the presence of schistosomal splenomegaly

Abstract Purpose: To evaluate a possible relationship between the size of the spleen and values of circulating blood elements in patients with schistosomatic splenomegaly. Methods: ixty one patients with hepatosplenic schistosomiasis mansoni underwent a clinical exam and peripheral venous blood was collected for a hemogram. The erythrocyte, hemoglobin, hematocrit, leukocyte, and platelet values were determined. All patients underwent abdominal ultrasound to measure the spleen. The hematological test results were compared to the size of the spleen. Results: The size of the spleen varied from 14.0 to 28.4 (19.9 ± 3.7) cm according to the ultrasound image. Thrombocytopenia was observed 58 (95%) patients, leukopenia in 55 (90%) patients, and anemia in 32 (52.4%) patients. Leukopenia was proportional to splenomegaly. Conclusion: Schistosomal splenomegaly leads to leukopenia in direct proportion to the size of the spleen.

Correlation between the values of circulating blood elements with the size of spleen in the presence of schistosomal splenomegaly.

PURPOSE: To evaluate a possible relationship between the size of the spleen and values of circulating blood elements in patients with schistosomatic splenomegaly. METHODS: ixty one patients with hepatosplenic schistosomiasis mansoni underwent a clinical exam and peripheral venous blood was collected for a hemogram. The erythrocyte, hemoglobin, hematocrit, leukocyte, and platelet values were determined. All patients underwent abdominal ultrasound to measure the spleen. The hematological test results were compared to the size of the spleen. RESULTS: The size of the spleen varied from 14.0 to 28.4 (19.9 ± 3.7) cm according to the ultrasound image. Thrombocytopenia was observed 58 (95%) patients, leukopenia in 55 (90%) patients, and anemia in 32 (52.4%) patients. Leukopenia was proportional to splenomegaly. CONCLUSION: Schistosomal splenomegaly leads to leukopenia in direct proportion to the size of the spleen.

Hepatoportal sclerosis related to the use of herbals and nutritional supplements. Causality or coincidence?

 Introduction and aim. Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. However, various pathophysiological mechanisms has been postulated, including chronic or recurrent infections and exposure to drugs or toxins. In this context, it appears to be of multifactorial etiology or resulting from a portal vascular endothelium aggression. It is important to consider whether the use of dietary supplements and herbs can trigger or contribute to the occurance of HPS. We report a possible association of HPS with the consumption of herbals and / or dietary supplements. MATERIAL AND METHODS: We describe two cases of HPS in patients without known etiology causes associated with this disease. RESULTS: Both patients were females who were diagnosed with HPS following the consumption of Herbalife® products and putative anorexigenic agents in the form herbals infusions. Image-based analysis and the assessment of the histopathological alterations found in the livers confirmed the diagnosis. The histopatological analysis of liver samples from both patients showed portal tracts enlarged by fibrosis with disappearance or reduction in the diameter of the portal vein branches. In many portal tracts, portal veins branches were replaced by aberrant thin-walled fendiforme vessels. The bile ducts and branches of the hepatic artery show normal aspects. CONCLUSION: After the exclusion of other etiologic factors and a comprehensive analysis of clinical history, consumption of Herbalife® products and anorexigenic agents was pointed-out as a puttative predisposing factor for the development of the disease.

Sodium caseinate induces increased survival in leukaemic mouse J774 model.

BACKGROUND: Acute myeloid leukaemia is a neoplastic disease of haematopoietic stem cells. Although there have been recent advances regarding its treatment, mortality remains high. Consequently, therapeutic alternatives continue to be explored. In the present report, we present evidence that sodium caseinate (CasNa), a salt of the principal protein in milk, may possess important anti-leukaemic properties. MATERIALS AND METHODS: J774 leukaemia macrophage-like cells were cultured with CasNa and proliferation, viability and differentiation were evaluated. These cells were also inoculated into BALB/c mice as a model of leukemia. RESULTS: We demonstrated that CasNa inhibits the in vitro proliferation and reduces viability of J774 cells, and leads to increased survival in vivo in a leukaemic mouse model. CONCLUSION: These data indicate that CasNa may be useful in leukaemia therapy.

Non-clinical safety studies of IMT504, a unique non-CpG oligonucleotide.

IMT504 is a non-CpG 24-mer oligodeoxynucleotide (ODN) with immunomodulatory as well as tissue repair activity. IMT504 has been previously proven to be effective in animal models of vaccine potency, chronic lymphocytic leukemia, tissue regeneration, and sepsis. Here, we assessed the safety, including pharmacokinetics and toxicity studies in rats and monkeys, of IMT504 in a single- or repeated-dose administration by the subcutaneous (SC) or intravenous (IV) routes. In rats, the maximum tolerated dose was determined to be 50 mg/kg when administered SC. Adverse effects at 50 mg/kg were mild and reversible liver injury, revealed as lobular inflammation, focal necrosis, and small changes in the transaminase profile. Dose-dependent splenomegaly and lymphoid hyperplasia, most probably associated with immune stimulation, were commonly observed. Rats and monkeys were also IV injected with a single dose of 10 or 3.5 mg/kg, and no adverse effects were observed. Rats injected IV with 10 mg/kg showed a transient increase in spleen weight, together with a slight increase in the marginal zone of the white pulp and in leukocyte count 2 days post-administration. In monkeys, this dosage caused slight changes in total serum complement and leukocyte count on day 14. No adverse effects were observed at 3.5 mg/kg IV in rats or monkeys. Therefore, this dose was defined as the "no observed adverse effect level" for this route. Furthermore, repeated-dose toxicity studies were performed in these species using 3.5 or 0.35 mg/kg/day IV for 6 weeks. A transient increase in the spleen and liver weight was observed at 3.5 mg/kg/day only in female rats. No changes in clotting time and activation of the alternative complement pathway were observed. The toxicity profile of IMT504 herein reported suggests a dose range in which IMT504 can be used safely in clinical trials.

Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis in late adulthood.

We describe a case of hemophagocytic lymphohistiocytosis related to visceral leishmaniasis in late adulthood. Because clinical features of visceral leishmaniasis can mimic those of hemophagocytic lymphohistiocytosis, diagnosing leishmaniasis as the underlying etiology can be quite challenging. In our case, treatment with amphotericin B resulted in a dramatic resolution of clinical abnormalities.

Linfoma esplénico de zona marginal como causa de esplenomegalia masiva: reporte de caso clínico y revisión de la literatura / Splenic marginal zone lymphoma as cause for massive splenomegaly: a clinical case and literature review

Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.

Visceral leishmaniasis in a captive crab-eating fox Cerdocyon thous.

Visceral leishmaniasis (VL) is a zoonotic disease with worldwide distribution. The crab-eating fox (Cerdocyon thous) is considered a wild reservoir of many zoonotical diseases, particularly VL. This study reported the presence of Leishmania infantum amastigotes in different organs of one captive C. thous found dead in a zoo. This animal was positive by the indirect fluorescence antibody test and had many clinical signs of VL. Intracellular amastigote forms of L. infantum were seen in neutrophils and macrophages in sample tissues from skin, lymph nodes (popliteal, submandibular, prescapular, and mesenteric), spleen, and liver. The numbers of positive cells and intracellular parasites were higher in macrophages than in neutrophils. In addition, polymerase chain reaction demonstrated extensive distribution of Leishmania DNA in C. thous tissues from multiple organs. The presence of intracellular amastigotes in neutrophils and macrophages as well as DNA of the parasite in tissues, specifically skin demonstrate that this crab-eating fox is an adequate host for L. infantum and reinforce the importance of VL for symptomatic wild canids kept in captivity in endemic areas.

Characterization of a murine lung adenocarcinoma (LAC1), a useful experimental model to study progression of lung cancer.

Lung cancer is one of the most important avoidable causes of death around the world, the most widespread carcinoma, with a very poor prognosis, and is the leading cause of cancer death in both developed and developing countries. We report morphological and biological behavior characteristics of a tumor that arose in only one BALB/c mouse of an experimental group treated with urethane, a chemical lung-tumorigenic agent. Morphological and immunochemical analysis indicated phenotypic compatibility with a lung adenocarcinoma. The tumor was named LAC1 (lung adenocarcinoma 1). Implant success in eight LAC1-bearing mice generations was 100%, with a fast evolution (58 survival days) and good metastatic capacity (41% of animals with metastases). The tumor induced a paraneoplastic syndrome characterized by anemia, neutrophilia, cachexia, splenomegaly and thymic atrophy. The lymphoproliferation to Con A was altered in tumor-bearing mice. This lung adenocarcinoma may be a useful experimental model for studying tumor progression, paraneoplastic syndromes and immunology in carcinogenic studies.

Malformación de abernethy tipo 1: presentación de un caso clínico y revisión de la literatura / Type-1 abernethy malformation: a clinic case presentation and review of literature

Se presenta caso de preescolar masculino de 5 años con antecedente de hepatoesplenomegalia desde el año de vida, quien consultó con clínica de hematuria, se realizó ecografía abdominal con hallazgos: de imágenes hipoecoicas difusas en el parénquima hepático, dificultad para valorar el sistema porta, esplenomegalia. Eco Doppler del sistema venoso portal presencia de anomalía vascular portal, no se observo porta principal, se realizó Angiotac multicorte del sistema arterial venoso portal y mesenterico que confirmo Agenesia de la vena Porta. Se diagnóstico malformación de Abernethy tipo I. Las malformaciones del sistema venoso abdominal son alteraciones vasculares raras. El primer acontecimiento de la ausencia congénita de la vena porta viene dado por un shunt cava mesenterico, los shunts portocava (SPC), son malformaciones infrecuentes descritas por Abernethy en 1973, se clasifican en dos grupos según la presencia tipo (II) o ausencia de la vena porta tipo (I). La malformación de Abernethy tipo I usualmente se relaciona a otras anomalías congénitas tales como: defectos cardiacos, atresia de vías biliares y poliesplenia, más frecuentes en el sexo femenino; en varones puede no encontrarse anomalías congénitas asociadas...

A 5-year old preschool male case is presented, with antecedent hepatosplenomegaly since one year old, who attended clinic consult with haematuria; abdominal echography was performed with the following findings: diffuse hypoecoic images on liver parenchyma, difficulties to assess the portal system, splenomegaly. Echo-Doppler of portal vein system evidenced the presence of portal vascular abnormality, no main portal vein was observed. A Multislice CT Angiography of the mesenteric and portal arterial-venous system was performed, which confirmed portal vein agenesis. Type-I Abernethy Malformation was diagnosed. The abdominal venous system’s malformations are rare vascular disturbances. The first event expressed by the portal vein congenital absence is evidenced by a caval-mesenteric shunt. Porta-Caval Shunt (PC-Shunts) are uncommon malformations described by Abernethy in 1973 that are classified into two groups as per the presence -Type II- or the absence -Type I- of portal vein. Type-I Abernethy Malformation is usually connected with other congenital abnormalities such as: heart defects, biliary tract atresia, and polysplenia, which are more frequent in females. Associated congenital abnormalities could not be found in males...

Leucemia de células velludas en el embarazo: Caso clínico / Hairy cell leukemia during pregnancy: Report of one case

Hairy cell leukemia (HCL) is a rare chronic B cell lymphoproliferative disorder that affects mostly men. It usually presents with pancytopenia, splenomegaly and bone marrow infiltration, without lymphadenopathy. Diagnosis is based on the presence of mononuclear cells with cytoplasmic projections in a blood smear, the typical bone marrow infiltration pattern and the immunophenotypic profile. HCL occurs seldom in young women and even more exceptionally during pregnancy. We report a 31-year-old woman in whom a splenomegaly was detected during routine prenatal care. Pancytopenia with 25 percent of hairy cells was found in her blood count. The patient was subjected to an open splenectomy and had an uneventful pregnancy. After two years of follow up, she has a normal blood count and has not required chemotherapy.

[Hairy cell leukemia during pregnancy: report of one case]. / Leucemia de células velludas en el embarazo: Caso clínico.

Hairy cell leukemia (HCL) is a rare chronic B cell lymphoproliferative disorder that affects mostly men. It usually presents with pancytopenia, splenomegaly and bone marrow infiltration, without lymphadenopathy. Diagnosis is based on the presence of mononuclear cells with cytoplasmic projections in a blood smear, the typical bone marrow infiltration pattern and the immunophenotypic profile. HCL occurs seldom in young women and even more exceptionally during pregnancy. We report a 31-year-old woman in whom a splenomegaly was detected during routine prenatal care. Pancytopenia with 25% of hairy cells was found in her blood count. The patient was subjected to an open splenectomy and had an uneventful pregnancy. After two years of follow up, she has a normal blood count and has not required chemotherapy.

Pathological findings associated with experimental infection by Trypanosoma evansi in cats.

Five cats were experimentally inoculated with Trypanosoma evansi in order to evaluate the pathological changes induced by this protozoan infection. Clinical signs observed included vomiting, diarrhoea, hyperthermia, weight loss, facial oedema, corneal opacity, lymphadenopathy and hindlimb instability. Reduction in hematocrit was observed from 7 days post-infection (dpi) (P<0.05). One cat died at 40 dpi and the other four cats were humanely destroyed. Necropsy examination was performed in two cats at 56 dpi and two cats at 120 dpi. Gross findings in all cats included generalized muscle atrophy, pale mucosae, icterus of the subcutaneous and serosal tissue and the intima of arteries, lymphadenopathy and splenomegaly. Other findings included corneal opacity, subcutaneous oedema (mainly of the head) and hydropericardium. Trypomastigotes of T. evansi were observed in impression smears prepared from the aqueous humor. Microscopically, there was lymphoid hyperplasia of the spleen and lymph nodes. The animals with corneal opacity had mild corneal oedema and accumulation of fibrin and inflammatory cells (neutrophils and plasma cells) in the anterior chamber. Similar inflammatory cells infiltrated the iris, ciliary body, corneoscleral limbus and conjunctiva.

Anomalía de Pelger-Huet: reporte de un caso / Pelger-huet anomaly: a case report

La anomalía de Pelger-Huet se observa una limitación de la segmentación nuclear de los granulocitos. La anomalía fue descrita por primera vez por Pelger en 1928, quien consideró que constituía una manifestación de tuberculosis. Huet consideró que la anomalía sería hereditaria y se transmitiría en forma autonómica dominante. Los individuos afectados rara vez presentan neutrófilos o eosinófilos con más de dos lóbulos. En los heterocigotos, el núcleo de los neutrófilos es no segmentado, con forma de pesa o bilobulado. En los homocigotos, la gran mayoría de los neutrófilos presentan núcleos redondos. Esta anomalía afecta aproximadamente a uno de cada 6000 individuos. La migración celular puede estar levemente alterada, pero la función de los granulocitos es normal y los individuos con esta anomalía hereditaria no padecen efectos adversos. Se trata de Rn masculino quien a las pocas horas de vida presenta ictericia neonatal y dificultad respiratoria y hepatoesplenomegalia. Hallazgos paraclínicos incompatibilidad de grupo sanguíneo, reacción leucemoide 98000 globulos blancos, PCR (-), e hiperbilirrubinemia a predominio de la indirecta. Se indica fototerapia, Oxigeno, y antibioticoterapia a base de PNC, Amikacina y vancomicina, Se realiza serología para TORCHS la cual reporta negativa y valoración por hematología la cual reporta anomalía de Pelger-Huet. Se presenta este caso para dar a conocer la existencia de esta anomalía como causa de errores frecuenctes al momento de valorar la hematología en procesos infecciosos y no infecciosos, que reportan reacciones leucemoides. Es importante que tanto el médico tratante como el paciente, esten en conocimiento de esta anomalía sanguínea, para valorar de forma adecuada el hemograma en posteriores oportunidades.

Evaluation of splenomegaly in the hepatosplenic form of mansonic schistosomiasis.

This study was aimed to evaluate splenomegaly in patients with the hepatosplenic (HS) form of mansonic schistosomiasis (MS), analyzing the size and weight of the spleen and their relationships with patients' gender and age. Between October, 1993 to July, 1998, 78 patients with the HS form of MS had undergone splenectomy as treatment of choice for bleeding due to portal hypertension, at Hospital das Clínicas, Pernambuco, Brazil. By means of abdominal palpation, the excess spleen felt below the left costal edge was measured, and the weight was obtained after splenectomy along with the histopathological analysis. Liver biopsy was performed intraoperatively in order to confirm MS and to rule out other liver diseases. The mean age of the 78 patients were 45 years and 41 of them (53%) were female. The average spleen weight was 912g and the mean spleen size palpable below the left costal edge was 9.1cm. There was a positive relationship between size and weight (p<0.001). Spleen weight and size were larger in males (p=0.007 and p=0.001, respectively). An inverse correlation between age and spleen weight was observed (p<0.001). A classification based upon spleen weight showed 53% of patients presenting a moderate (501-1000g) and 33% a severe (>1001g) splenomegaly. As for the spleen size, the classification showed 64% of patients presenting moderate (4.1-10cm below the left costal edge) and 21% severe (>10cm) splenomegaly. In conclusion, splenomegaly may be considered a key physical finding in patients with HS form of MS, and we found a good correlation between the spleen sizes clinically evaluated with its weight. The majority of cases presents a moderate to severe splenomegaly and spleen size is larger in men and it seems to decrease with aging.

Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy.

A 5-year-old male with Gaucher's disease type 3 developed progressive mesenteric and mediastinal lymphadenopathy over 12 months, despite enzyme replacement therapy, contributing to the development of a protein-losing enteropathy. These complications are unique, indicating poorly accessible, differentially responsive compartments in patients with Gaucher's disease who are receiving enzyme therapy.