SPLENIC PATHOLOGY

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CHAPTER 9 SPLENIC PATHOLOGY

Spenunculus / accessory spleens
Splenic ‘cysts’
Splenogonadal fusion

Splenectomy for hypersplenism

Immune thrombocytopenic purpura (ITP)
Hemolytic anemia [congenital (eg spherocytosis) or acquired]
Congestive splenomegaly
Other miscellaneous splenic disease

Storage disease
Infection
Involvement by malignancy
Sickle cell disease
Spleen in immunodeficiency

INTRODUCTION

• Spleen may be involved with many systemic processes

image most are covered in other sections

• Lesions addressed here are those splenic conditions not encountered elsewhere in the text
• In general, splenic pathology forms a small component of most pediatric surgical pathology workload

image spleen may also be removed following trauma

SPENUNCULUS / ACCESSORY SPLEENS (Fig 9.1)

• Often removed as incidental findings

image usually asymptomatic

• May enlarge following splenectomy for other indication
• No specific histological features
image

Fig 9.1 Photomicrograph of a splenunculus, demonstrating an encapsulated nodule of otherwise unremarkable splenic tissue.

SPLENIC ‘CYSTS’

• The majority of nonparasitic splenic cysts in children are squamous lined epidermoid cysts (Czauderna et al 2006)
• Other rarer types include splenic pseudocyst or mesothelial cyst

image hemangioma or lymphangioma are also described

– often diagnosed as ‘cysts’ preoperatively

Pseudocysts

• Dense fibrosis tissue in wall

image no identifiable epithelial lining

• Usually contain blood and necrotic debris

Epithelial cysts (Figs 9.2–9.5)

• Usually solitary
• Usually present in childhood

image incidentally
image mass lesion

• Cyst wall lined by bland epithelium

image squamous
image columnar
image cuboidal

– possibly derived from epithelial inclusions

image

Fig 9.2 Macroscopic photograph of a spleen resected with a simple epithelial cyst; residual clear/yellow fluid is seen.

image

Fig. 9.3

image

Fig. 9.4

image

Figs 9.3–9.5 Photomicrographs of simple epithelial splenic cysts, demonstrating lining which is focally attenuated and predominantly simple squamous in type, with surrounding fibrosis and otherwise normal splenic tissue.

Parasitic

• Echinococcus infection

image rare in UK

SPLENOGONADAL FUSION

• Usually incidental finding
• Histologically unremarkable splenic tissue
• May be a continuous connection or discontinuous (see ‘gonads’ section)

SPLENECTOMY FOR HYPERSPLENISM

• Relatively common indication for open or laparascopic splenectomy in childhood
• Specific histological features are listed below

image note that splenic infarcts may represent either:

– a vasculitic process
– non-specific finding secondary to splenomegaly of other cores

• White pulp changes are variable

IMMUNE THROMBOCYTOPENIC PURPURA (ITP) (Figs 9.6, 9.7)

• Spleen is commonly removed for therapeutic indications in ITP in childhood (Hayes et al 1985, Chang et al 1993)
• Prominent lymphoid follicles
• Red pulp prominence

image dilated sinuses
image numerous macrophages

– often foamy macrophages
– platelet destruction by cordal macrophages

• Myeloid metaplasia and extramedullary hematopoiesis

image especially megakaryocytes

• Note: numerous foamy macrophages in the spleen are reported with:

image normal variant
image ITP
image Gaucher disease
image Niemann–Pick disease
image Tay–Sachs disease
image CGD
image thalassemia
image hyperlipidemia

image

Fig. 9.6

image

Figs 9.6–9.7 Photomicrographs of a spleen removed from a patient with idiopathic thrombocytopenic purpura (ITP), demonstrating red pulp expansion with numerous macrophages present, and prominent lymphoid follicles.

HEMOLYTIC ANEMIA [CONGENITAL (EG SPHEROCYTOSIS) OR ACQUIRED] (Fig 9.8)

• Spleen is commonly removed for therapeutic indications in hemolytic anemia in childhood (Chang et al 1993)
• Prominent red pulp
• Congested cords

image widened by sequestered red blood cells

• Prominent sinus lining cells
• Hemosiderin deposition (variable)
• Erythrophagocytosis

image rarely seen on routine sections

• Autoimmune (acquired) hemolytic disorders show prominent intrasinusoidal phagocytosis
• Hereditary spherocytosis may also show white pulp expansion
• Neutrophils may also be present
image

Fig 9.8 Photomicrograph of a spleen removed from a patient with congenital spherocytosis, demonstrating prominent red pulp with markedly congested cords.

CONGESTIVE SPLENOMEGALY

• Usually secondary to portal hypertension
• Dilated veins and sinuses
• Capsular thickening
• Hemosiderin-laden macrophages
• Patchy red pulp fibrosis

OTHER MISCELLANEOUS SPLENIC DISEASE

STORAGE DISEASE (Fig 9.9)

• Features specific to each metabolic disease
• Splenic involvement not usually major features except:

image Gaucher disease
image Niemann–Pick disease
image sea-blue histiocyte disease

• Large collections of macrophages expand red pulp

image Contain abnormal storage material

image

Fig 9.9 Photomicrograph of a spleen from a patient with splenomegaly due to Gaucher disease, demonstrating large numbers of cells containing abnormal storage material.

INFECTION (Figs 9.10, 9.11)

• Hydatid cysts due to Echinococcus infection
• Infections, mainly in immunocompromised:

image Mycobacterial infection
image Histoplasmosis
image Cryptococcosis
image Leishmaniasis
image malaria

image

Fig. 9.10

image

Figs 9.10–9.11 Photomicrographs of spleens with granulomatous inflammation, due to presumed mycobacterial infection in an immunocompromised child with HIV-infection, and a child with BCG-osis, respectively.

INVOLVEMENT BY MALIGNANCY

• Usually lymphoma / leukemia (Figs 9.12, 9.13)
• Almost all types may theoretically affect the spleen

image most rare in childhood

– more commonly juvenile myelomonocytic leukemia and Hodgkin disease in this group

image red pulp cords and sinusoids contain blasts

• Primary splenic malignancy almost unheard of in childhood
image

Fig. 9.12

image

Figs 9.12–9.13 Photomicrographs of a spleen from a child with juvenile myelomonocytic leukemia (JMML), demonstrating expansion by a population of abnormal, malignant hematolymphoid cells.

SICKLE CELL DISEASE (Fig 9.14)

• Splenomegaly due to massive sequestration of sickled red cells

image with time develops infarcts and hyposplenism
image chronic hemosiderin deposition

image

Fig 9.14 Photomicrograph of a spleen from a child with sickle cell disease, demonstrating red pulp congestion due to accumulation of sickled red cells within the sinusoids and cords.

SPLEEN IN IMMUNODEFICIENCY

• Most immunodeficiencies show reduced white pulp

image splenic lymphoid hypoplasia

• Well described in Wiskott–Aldrich Syndrome (Fig 9.15)

image spleens from WAS patients showed a significant depletion of the total white pulp (Vermi et al 1999)
• Superimposed opportunistic infections
• In HIV infection features include (Klatt et al 1987)

image lymphoid depletion
image proliferation of histiocytes
image plasma cell/immunoblastic reaction
image

Fig 9.15 Photomicrograph of a spleen from a child with Wiskott–Aldrich syndrome, demonstrating marked white pulp depletion.

REFERENCES

Chang CS, Li CY, Cha SS. Chronic idiopathic thrombocytopenic purpura. Splenic pathologic features and their clinical correlation. Arch Pathol Lab Med. 1993;117:981-985.

Chang CS, Li CY, Liang YH, Cha SS. Clinical features and splenic pathologic changes in patients with autoimmune hemolytic anemia and congenital hemolytic anemia. Mayo Clin Proc. 1993;68:757-762.

Czauderna P, Vajda P, Schaarschmidt K, et al. Nonparasitic splenic cysts in children: a multicentric study. Eur J Pediatr Surg. 2006;16:415-419.

Hayes MM, Jacobs P, Wood L, Dent DM. Splenic pathology in immune thrombocytopenia. J Clin Pathol. 1985;38:985-988.

Klatt EC, Meyer PR. Pathology of the spleen in the acquired immunodeficiency syndrome. Arch Pathol Lab Med. 1987;111:1050-1053.

Vermi W, Blanzuoli L, Kraus MD, et al. The spleen in the Wiskott–Aldrich syndrome: histopathologic abnormalities of the white pulp correlate with the clinical phenotype of the disease. Am J Surg Pathol. 1999;23:182-191.

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