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Platelet Disorders Douglas Triplett
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
In 5 to 20% of patients with ITP, splenectomy produces little or no lasting benefit. Unfortunately, there is no way of predicting this prior to the operation. The value of splenic sequestration studies is controversial, and currently they are not relied upon. Accessory spleens are mentioned frequently as the cause of failure of splenectomy. Most accessory spleens will be found in the area of the splenic pedicle and can be easily recognized and removed by the surgeon. However, occasionally, distant sites such as lower pelvis, lungs, and liver are encountered, and in these instances the spleen is not easily identified. Rupture of the spleen at surgery may produce splenosis — multiple small areas of splenic tissue in the peritoneum, representing implants of deposited cells. Surgical removal of the accessory spleen may in some instances result in a permanent remission, although not in all cases. Presumably, an exacerbation of the patient’s immune process is responsible for recurrent thrombocytopenia in the majority of cases. In recent years, a number of immunosuppressive drugs have been used in cases of refractory ITP. These have included 6-mercaptopurine, azathioprine, alkylating agents such as Cytoxan® and chlorambucil, and vinka alkaloids such as vincristine.256
The Lymphoid System
Published in Pritam S. Sahota, James A. Popp, Jerry F. Hardisty, Chirukandath Gopinath, Page R. Bouchard, Toxicologic Pathology, 2018
Accessory spleens, sometimes referred to as ectopic spleens or splenic nodules, are occasionally seen in cynomolgus monkeys. These may be embedded in or attached to the pancreas. We have seen such ectopic spleens in up to 15% of cynomolgus monkeys used in toxicology studies. Similar structures are seen in man and have been called “splenculi,” and according to Han et al. (1997), can be found in 25% of human autopsies. These authors also indicate that in pathologic conditions requiring splenectomy, recrudescence of the condition may be due to the presence of small accessory spleens. Also, in the pig, the gastrosplenic ligament may contain accessory spleens.
The spleen and lymph nodes
Published in Michael Gaunt, Tjun Tang, Stewart Walsh, General Surgery Outpatient Decisions, 2018
There is a need to differentiate between splenosis and accessory spleens. Accessory spleens are found at the hilum of the spleen and omentum; number fewer than 10; and have hilar vessels with normal splenic architecture. Implantation splenosis tends to number more than 20; there is a history of trauma; they are scattered over the peritoneum; and do not have a co-ordinated circulation.
Intrapancreatic accessory spleen mimicking pancreatic NET: can unnecessary surgery be avoided?
Published in Acta Clinica Belgica, 2021
Elisabeth Vandekerckhove, Eline Ameloot, Anne Hoorens, Kathia De Man, Frederik Berrevoet, Karen Geboes
Abdominal splenosis is the spontaneous transplantation of splenic tissue to unusual sites. It can occur in any intraperitoneal or extraperitoneal location, nonetheless it is most common in the peritoneum, omentum and mesentery. This situation usually occurs after splenic trauma such as automobile accidents, stab or gunshot wounds and surgery [1]. When there is no history of trauma, another explanation is an accessory spleen, a congenital disorder that develops around the 5th week of gestation with fusion failure of the splenic base in the dorsal mesogastrium during embryological splenic development. An accessory spleen is frequent: autopsy reports revealed an accessory spleen in 10–30% of patients. One study found an abdominal accessory spleen in 16% of 1000 patients who underwent a CT scan. In 80% of these cases, the accessory spleen was located near the splenic hilum, the second most common site was the tail of the pancreas (17%) [2].
Three cases of molecularly confirmed Knobloch syndrome
Published in Ophthalmic Genetics, 2020
Irina Balikova, Nuri Serdal Sanak, Depasse Fanny, Guillaume Smits, Julie Soblet, Elfride de Baere, Monique Cordonnier
Patient 2 had important extraocular anomalies – kidney atrophy, accessory spleen and showed delayed skin cicatrisation. The end-stage renal disease in a relatively young age could be related to the mutation in COL18A1, since at least in the col18a1 knockout mice there are ultrastructural changes in the kidney (8) which might lead to altered renal function and stress tolerance predisposing to renal insufficiency. In addition, Col18a1 knockout mice have elevated creatinine levels (9) which also indicates that Col18a1 affects renal filtration and function. However, terminal end-stage renal disease and renal atrophy related to renal insufficiency can be secondary to the elevated blood pressure, vascular defects or diabetes. Another observation is the difficult skin healing which can be related to the COL18A1 insufficiency. The COL18A1 gene is expressed in the skin (10) and takes part in the basal membrane. The role of COL18A1 in modulating the wound healing has been shown by Seppinen et al. (11). The authors conclude that the lack of Col18a1 accelerates wound healing while the overexpression of the endostatin domain leads to delayed healing in mice. However, in oral mucosa, the lack of Col18a1 leads to a dramatic decrease in the markers of keratinisation (12). In addition, Maeba et al. recently showed that COL18A1 has a role in the dermal-epidermal junction during wound healing (13). The genetic defect in patient 2 leads to loss of function of COL18A1 including the endostatin isoform. Possibly this causes delayed epithelialisation. Again, this has to be further investigated since systemic (hypertension, dyslipidemia, kidney insufficiency) and local (venous insufficiency in lower limbs) factors affect wound healing as well. Accessory spleen is present in 11% in the normal population and its link with the syndrome is not certain (14). The mental development of the patients ranged from normal to learning difficulties. None of the patients had epilepsy or severe mental problems.
Second-line and beyond: treatment options for primary persistent and chronic immune thrombocytopenia
Published in Platelets, 2020
Samuel Deshayes, Bertrand Godeau
– Splenectomized patients may show response to previously inefficient treatments, and a new short course of steroids should be tried before proposing other options. An accessory spleen must be sought, even if the results of removal of an accessory spleen are often disappointing [121].