A Cadaveric Study on Morphometric Features of Spleen and Splenomegaly with Accessory Spleen in Hilum

Background: Anatomical knowledge regarding the external morphology of the spleen is essential for surgical intervention and radiological diagnosis. Splenomegaly is defined as pathologic enlargement of the spleen measured by size or weight. A normal spleen has a craniocaudal length of no more than 12 cm and weighs less than 200 g. It is surrounded by a thin capsule. The spleen is usually not palpable unless it is enlarged; therefore, a palpable spleen is almost always abnormal. At times the spleen may be difficult to palpate, but dullness to percussion during inspiration in the area of the lower left intercostal space in the left anterior axillary line suggests splenic enlargement. Massive splenomegaly, weight >1000 g usually occurs in lymphoma, myeloproliferative disorders, visceral leishmaniasis, and malaria. Materials and Methods: This study was conducted in different medical institutions, to find morphometric features, spenomegaly in cadaver during routine anatomy dissection as part of curriculum, 100 cadavers were observed to find out splenomegaly. Results: Out of 100 spleens studied, 81 cases wedge shaped spleen was the most common, followed by 12 tetrahedral shaped spleens and 7 oval shaped spleens. Average weight of the spleen was 175g. Average length of the spleen was 11.64cm, Average breadth of the spleen was 7.3cm and average thickness of spleen was 3.6cm. Out of 100 cadavers observed only one cadaver observed with massive splenomegaly with one accessory spleen in hilum. The spleen weight was 875gm, length was 18.15 cm, width was 8.65cm, thickness was 5.75cm and extended upto 7 rib and it is easily palpable below the rib cage from lumbar aspect. The cadaver was male and age around 55 years. Conclusion: The morphometric knowledge of spleen will helpful for surgeons and for understanding deceases related spleen. The knowledge of splenomegaly is important in finding splenic disorders and accessory spleen information helpful in understanding embryonic development of spleen. KEY WORDS: Splenomegaly, Spleen, Hilum of Spleen, Accessory spleen.

Case Report: Intravenous Pentamidine Rescue Treatment for Active Chronic Visceral Leishmaniasis in an HIV-1 Infected Patient

The management of visceral leishmaniasis (VL) in HIV-infected patients is complex because of high mortality rates, toxic drug-related side effects, and a high risk of treatment failure and relapse. We report a case of active chronic VL in an HIV-1-infected woman presenting multiple secondary VL episodes over 7 years leading to massive splenomegaly and blood transfusion–dependent anemia despite several treatment courses and secondary prophylaxis. The patient was finally successfully treated with rescue treatment based on intravenous pentamidine. One year after discontinuation of pentamidine the patient presented complete clinical and parasitological response. In patients with active chronic VL, rescue treatment with intravenous pentamidine can be effective and should be considered as rescue treatment.

STUDY OF SPLENOMEGALY IN CHILDREN.

Splenomegaly is common clinical finding in pediatric practice. Splenic enlargement occurs when the spleen is increased by cells or tissue components or by vascular engorgement. Various etiologies can cause splenomegaly. The spleen is rarely the primary site of a disease. Splenomegaly is classified according to the length palpable below the costal margin as mild: <3 cm, moderate: 4-7 cm & massive: >7 cm. Severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages. Only in the age group of 1 to 5 years females predominated as compared to males. In present study there is obvious male predominance as male: female ratio is 1.8:1. This difference could be due to more priority to male child to seeking medical care with such chronic illness.Present study also suggested that severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages.

NON-CIRRHOTIC PORTAL FIBROSIS CAUSING PORTAL HYPERTENSION

Portal hypertension is more prevalent in patients with liver cirrhosis and occurs infrequently in those without liver cirrhosis. Non-cirrhotic portal brosis (NCPF) and extrahepatic portal venous obstruction (EHPVO) are the two most common causes of non-cirrhotic portal hypertension. Unlike EHPVO, NCPF does not cause thrombosis of the extrahepatic portal vein. Sclerosis of the portal vein's medium and small branches occurs in NCPF. In NCPF, the hepatic venous pressure gradient (HVPG) is normal, in contrast to cirrhosis, where it is increased. Additionally, NCPF is referred to as non-cirrhotic intrahepatic portal hypertension (NCIPH), idiopathic portal hypertension, hepatoportal sclerosis, and benign intrahepatic portal hypertension. It is a disease with an unknown etiology that primarily affects middle-aged males and females and manifests as hematemesis and massive splenomegaly

POS0418 SPLENIC EXTRAMEDULLARY HEMATOPOIESIS IS OMNIPRESENT AND CORRELATES WITH DISEASE SEVERITY IN THE LUPUS NZB/W F1 MURINE MODEL

Background:Extramedullary hematopoiesis (EMH) is increasingly recognized as an integral component of systemic inflammatory diseases; compared to their bone marrow counterparts, hematopoietic progenitors of EMH have an enhanced role in target organ damage1,2. We have found that β-glucan -a non-specific inducer of reprograming of innate immunity- results in dramatic EMH with marked increase in Long-Term (LT)-HSCs, massive splenomegaly and worsening of nephritis in the NZB/W F1 lupus murine model (unpublished data).Objectives:To investigate EMH’s time course and contribution to inflammatory target-organ damage (kidney) in the NZB/W F1 lupus murine model.Methods:Spleens and kidneys were isolated from female NZB/W F1, at pre-nephritic stage (3-month-old) and nephritic stage (>6-month-old), and age/sex matched C57BL/6 (WT controls). Single-cell suspensions of spleens were analyzed by flow cytometry for Hematopoietic Stem and progenitor cells (HSPCs) phenotyping. Formalin-fixed and paraffin-embedded sections of spleens and kidneys were stained with conventional histological stains (H&E, Silver, Trichrome Masson). Spleens were histologically assessed for the presence of ΕΜΗ and kidneys were assessed for activity and chronicity through the NIH Lupus nephritis scoring system.Results:Histological analysis revealed that NZW/B F1 mice at the nephritic stage display massive splenomegaly with concomitant expansion of the red pulp, increased presence of megakaryocytes and disorganized splenic architecture. This is further corroborated by the flow cytometry analysis which demonstrated a significant increase of all HSPCs subsets (Long-term/Short-term Hematopoietic Stem Cells and Multipotent progenitors) compared to the C57BL/6 WT controls at nephritic stage. The degree of HSPC expansion and splenic architecture disorganization correlates strongly with the activity of lupus nephritis as quantified by the NIH scoring system. Of note, evidence of splenic EMH were present even in 3-month-old animals before overt nephritis ensues.Conclusion:Extramedullary hematopoiesis is present before overt nephritis and is dramatically expanded at the nephritic stage of the NZW/B F1 mouse model. The degree of EMH positively correlates with the severity of lupus nephritis. These data support a pathogenic role of EMH, and spleen derived HSPCs, in driving lupus nephritis.References:[1]Regan-Komito, D., Swann, J.W., Demetriou, P., Cohen, E.S., Horwood, N.J., Sansom, S.N., Griseri, T., 2020. GM-CSF drives dysregulated hematopoietic stem cell activity and pathogenic extramedullary myelopoiesis in experimental spondyloarthritis. Nature Communications 11, 155.[2]Griseri, T., McKenzie, B.S., Schiering, C., Powrie, F., 2012. Dysregulated Hematopoietic Stem and Progenitor Cell Activity Promotes Interleukin-23-Driven Chronic Intestinal Inflammation. Immunity 37, 1116–1129.Acknowledgements:This project has received funding from the European Research Council (ERC) under the European Union’s Horizon 2020 research and innovation programme (grant agreement No 742390)Disclosure of Interests:None declared

Massive Splenomegaly: A Rare Presentation of Megaloblastic Anemia

Megaloblastic anemia is a common disorder with various manifestations. Of the many causes, cobalamin or folate deficiency can eventuate into megaloblastic anemia. It can lead to pancytopenia and mild to moderate splenomegaly, but massive splenomegaly rarely seen in this situation. We describe a 39-yearold woman with marked enlargement of the spleen and pancytopenia that was found to have megaloblastic anemia. The splenomegaly and blood count resolved 4 months after initiation of vitamin B12 therapy. It is important to know massive splenomegaly may occur in megaloblastic anemia, and although it is rare, bur can reversible with early treatment.