Answer to the question from page 469.

Prawidłowa odpowiedź: A.

Correct answer: A.

The identification of an enlarged organ does not pose any problems for specialists. However, experts do not provide the values that would attest to the decreased size of organs. For instance, this refers to the gallbladder, liver, pancreas and spleen(. The references do not provide much information about hyposplenia. This issue seems to have been thoroughly discussed by Görg et al.( The aim of their paper was to determine the correlations between impaired splenic function and its size. The authors arbitrarily assumed that the spleen smaller than 7 × 3 cm may be considered hypoplastic. The analyzed group consisted of 24 patients with various diseases (the most common was sickle-cell anemia – 7 cases). Functional hyposplenism was diagnosed using scintigraphy with damaged erythrocytes labeled with Tc99m or based on the presence of Howell-Jolly bodies within red blood cells. In 20 patients (83%), the spleen was smaller than 7 × 3 cm. What is more, the reduction of blood supply to the spleen, which was manifested with the lack of or scarce parenchymal flow, was observed in 88% of cases. There may be several causes of splenic dysfunction:

infiltration of the spleen by a pathological tissue (meta-stases, lymphoma, metabolic diseases or amyloidosis);

impaired blood supply to the spleen – narrowed or occluded splenic vessels (in the course of pancreatitis, splenic torsion or splenoptosis, vasculitis, thrombophilia and antiphospholipid syndrome);

graft-versus-host disease (rare cases).

None of the aforementioned pathologies were detected in the patient discussed in the quiz. What is more, no Howell-Jolly bodies were found within erythrocytes.

What is the cause of hyposplenia then? Is it idiopathic or age-related?

Usually after the age of 50, a range of organs undergo a gradual involution. This refers to the liver, kidneys, uterus, ovaries and spleen. Such a possibility is also mentioned in the publication discussed above(. This reason is possible in the patient analyzed in the quiz due to his age (65 years of age), the lack of pathological factors leading to hyposplenia, the lack of Howell-Jolly bodies within erythrocytes and the lack of susceptibility to infections. The disease-unrelated small spleen is also presented in two other cases: 82-year-old (fig. 2) and 80-year-old women (fig. 3). Görg(, the author mentioned above, assessed changes in the spleen and its surrounding tissues using contrast-enhanced ultrasound examinations (CEUS). According to his analyses, among 6 cases with indicated CEUS examination, there was also hyposplenia with impaired macrophage function.

Fig. 2

Fig. 3

As has already been mentioned, atrophic spleen in older age is not usually related to any pathology. In young adults and middle-aged patients, small spleen should raise suspicion of celiac disease. It is estimated that hyposplenism occurs in 25–75% of patients with celiac disease which may cause a serious infection, including sepsis. Such patients are therefore advised to get vaccinated against pneumococci and H. influenzae. Furthermore, hyposplenism in celiac disease may be concomitant with so-called, cavitating mesenteric lymph node syndrome characterized by a severe course of the disease. It appears that hyposplenism in celiac disease is reversible after instituting a gluten-free diet. On the other hand, it may show no response to such a diet, which consequently leads to the atrophy of the spleen(.