Spleen histology in children with sickle cell disease and hereditary spherocytosis: hints on the disease pathophysiology

Abstract

Hereditary spherocytosis (HS) and sickle cell disease (SCD) are associated with splenomegaly and spleen dysfunction in pediatric patients. Scant data exist on possible correlations between spleen morphology and function in HS and SCD. This study aimed to assess the histologic and morphometric features of HS and SCD spleens, to get possible correlations with disease pathophysiology. In a large series of spleens from SCD, HS, and control patients, the following parameters were considered: (i) macroscopic features, (ii) lymphoid follicle (LF) density, (iii) presence of perifollicular marginal zones, (iv) presence of Gamna-Gandy bodies, (v) density of CD8-positive sinusoids, (vi) density of CD34-positive microvessels, (vii) presence/distribution of fibrosis and smooth muscle actin (SMA)-positive myoid cells, and (viii) density of CD68-positive macrophages. SCD and HS spleens had similar macroscopic features. SCD spleens had lower LF density and fewer marginal zones than did HS spleens and controls. SCD also showed lower CD8-positive sinusoid density, increased CD34-positive microvessel density and SMA-positive myoid cells, and higher prevalence of fibrosis and Gamna-Gandy bodies. HS had lower LF and CD8-positive sinusoid density than did controls. No significant differences were noted in red pulp macrophages. By multivariate analysis, most HS spleens clustered with controls, whereas SCD grouped separately. A multiparametric score could predict the degree of spleen changes irrespective of the underlying disease. In conclusion, SCD spleens display greater histologic effacement than HS, and SCD-related changes suggest impaired function due to vascular damage. These observations may contribute to guide the clinical management of patients.

Gamna‐Gandy bodies in fine‐needle aspiration from abdominal splenosis: A clue to underlying portal hypertension

“…Most modern discussions of GGBS are found in the radiology literature. The magnetic resonance imaging appearance of GGBS and their association with portal hypertension has been widely discussed 4,6,20‐22 . However, descriptions of the morphology and clinical significance of GGBS are found largely in case reports and omitted in modern pathology textbooks 3 .…”

“…The spleen is the most common site for GGBS formation. Severe congestive splenomegaly secondary to portal hypertension and vascular injury secondary to sickle cell disease are the two most commonly reported underlying conditions 3‐7 . Abdominal splenosis is a well‐known sequela of traumatic or surgical splenectomy and can mimic peritoneal carcinomatosis 8,9 .…”

Telomerase-Positive Stem Cells in Adult Porcine and Adult Rat Spleens I. Totipotent Stem Cells

“…Spleen enlargement can also occur and is known as splenomegaly. Splenomegaly can be caused by obstruction of blood flow, underlying abnormality, infiltration, or antigenic stimulation [23][24][25][26][27][28][29][30].…”

Effect of titanium dioxide nanoparticles on the spleen of adult male albino rats: Histological and immunohistochemical study

“…Frequently, enclosed within the PALS are lymphoid follicles (LFs) which are composed of B cells and displace the central arteriole to a peripheral position. Primary and secondary LFs together display an outer marginal zone, composed of lymphocytes with abundant cytoplasm [13,18,19] . Red pulp is composed of venous sinuses filled with blood and cords of splenic tissue called splenic (Billroth's) cords.…”