Hypersplenism

Hypersplenism is excessive sequestration and destruction of circulating blood cells by an enlarged or hyperfunctioning spleen.

The characteristic clinical pattern is:

Splenomegaly
One or more peripheral cytopenias
Preserved or compensatory bone marrow production

Cytopenia can involve:

Platelets
Red cells
White cells

or all three cell lines.

Thrombocytopenia is often the most prominent abnormality because a normal spleen already sequesters a substantial fraction of circulating platelets.

Hypersplenism is a syndrome caused by another disorder, not a final diagnosis.

Important causes include:

Portal and congestive

Cirrhosis
Portal hypertension
Portal or splenic vein thrombosis

Haematological

Myelofibrosis
Lymphoma
Leukaemia
Haemolytic anaemia
Thalassaemia

Infectious

Malaria
Visceral leishmaniasis
Chronic systemic infection

Inflammatory

Felty syndrome

Storage disease

Gaucher disease

The spleen can become markedly enlarged and hyperfunctional in many of these conditions.

Patients can present with consequences of the enlarged spleen:

Left upper quadrant discomfort
Early satiety
Abdominal fullness
Mechanical pressure symptoms

or consequences of cytopenia:

Thrombocytopenia

Easy bruising
Mucosal bleeding
Prolonged bleeding

Anaemia

Fatigue
Dyspnoea
Tachycardia

Leukopenia or neutropenia

Recurrent bacterial infection

A patient can have substantial hypersplenism without spontaneous bleeding because platelet function and hepatic coagulation status also influence bleeding risk.

Initial evaluation should establish both the presence of cytopenia and the underlying disease.

Obtain:

Full blood count with differential
Peripheral blood film
Reticulocyte count
Liver function
Coagulation profile
Renal function
Haemolysis profile where relevant
Disease specific investigations according to clinical context

Ultrasound or CT defines splenic size and evaluates:

Portal vein
Splenic vein
Liver morphology
Lymphadenopathy
Focal splenic lesion

Bone marrow

Bone marrow examination is not required simply to prove hypersplenism.

It is appropriate when:

Haematological malignancy is possible
Pancytopenia remains unexplained
Marrow failure must be distinguished from peripheral sequestration
Myelofibrosis is suspected

A hypocellular failing marrow argues against uncomplicated hypersplenism as the sole explanation for cytopenia.

Treat the underlying cause

This is the first priority.

Examples include:

Portal hypertension treatment
Antimalarial treatment when infection is confirmed
Disease directed therapy for lymphoma or leukaemia
Myelofibrosis directed systemic therapy
Treatment of autoimmune disease

Do not perform splenectomy merely to normalise an abnormal full blood count when the underlying disease can be treated directly.

Blood product support

Transfuse according to clinical need, not simply to normalise laboratory values.

Platelet transfusion in severe hypersplenism can produce only transient increases because platelets are rapidly sequestered.

Reserve transfusion for:

Clinically significant bleeding
Major invasive procedure
Critical thrombocytopenia with a high bleeding risk

Indications for splenectomy

Consider splenectomy when hypersplenism produces clinically important morbidity despite appropriate treatment of the underlying disorder, such as:

Recurrent clinically significant bleeding from severe thrombocytopenia
Severe symptomatic anaemia or transfusion dependence caused predominantly by splenic sequestration or destruction
Recurrent infection from severe neutropenia
Painful or mechanically disabling splenomegaly
Recurrent splenic infarction
Rupture risk or previous rupture

The expected haematological benefit must be weighed against lifelong:

Infection risk
Thrombosis risk
Operative morbidity

Partial splenic embolisation

Partial splenic artery embolisation can reduce functional splenic mass without complete removal.

It is useful in selected patients who:

Have major portal hypertension
Are poor operative candidates
Need improvement in cytopenia

Complications include:

Severe post embolisation pain
Fever
Splenic infarction
Abscess
Portal vein thrombosis

Excessive embolisation increases major complication risk.

Portal hypertension

Splenectomy may improve hypersplenic thrombocytopenia but does not correct the underlying portal hypertensive physiology.

Patients with cirrhosis and very large spleens have an important postoperative risk of portal and splenic venous thrombosis, so surgery should not be undertaken solely for a mildly low platelet count.

Clinical toolsCalculators

BMI GCS EDD Parkland Formula

Corrected Sodium Corrected Calcium Anion Gap Creatinine Clearance Insulin Calculations qSOFA Wells PE Wells DVT CHA₂DS₂-VASc HAS-BLED CURB-65 Centor/McIsaac

Small Bowel Adenocarcinoma

Small bowel adenocarcinoma is a rare epithelial malignancy arising most commonly in the duodenum, followed by jejunum and ileum. Predisposing conditions include Crohn disease, coeliac disease, Lynch syndrome, familial adenomatous polyposis and Peutz Jeghers syndrome.

Curative treatment depends on complete surgical resection with adequate regional lymph node assessment. Node positive disease has a substantially worse prognosis and generally requires adjuvant chemotherapy.

Symptoms depend on site.

Jejunal and ileal disease commonly presents with intermittent obstruction, abdominal pain, anaemia, occult bleeding or weight loss.

Duodenal disease may produce vomiting, jaundice or gastric outlet symptoms depending on location.

An adult with unexplained recurrent small bowel obstruction without previous surgery should be investigated for a structural lesion, including malignancy.

CT enterography or contrast CT: define the primary lesion, regional lymphadenopathy and distant metastases.

Upper endoscopy or enteroscopy: obtain tissue when the lesion is endoscopically accessible.

Mismatch repair or microsatellite instability testing should be performed because it has prognostic and therapeutic implications.

Regional nodal staging is important. N1 disease means one or two positive regional nodes and N2 disease means three or more.

Surgery

Resectable jejunal or ileal adenocarcinoma requires segmental small bowel resection with en bloc regional mesenteric lymphadenectomy.

A tumour involving the terminal ileum or ileocaecal valve usually requires ileocaecal resection or right hemicolectomy with appropriate nodal clearance.

Duodenal cancers involving the second portion or ampullary region commonly require pancreaticoduodenectomy. Selected proximal first portion or distal third and fourth portion tumours may undergo segmental duodenal resection when an oncologically adequate margin and nodal dissection can be achieved.

Adjuvant treatment

Stage I disease is generally observed after complete resection.

Node positive stage III disease should receive approximately 6 months of adjuvant fluoropyrimidine based chemotherapy, preferably an oxaliplatin containing doublet in a fit patient. High risk stage II disease may also justify adjuvant treatment, particularly with T4 disease, perforation, inadequate nodal harvest, poor differentiation, lymphovascular invasion or positive or close margins.

A standard modified FOLFOX6 regimen every 14 days is:

Oxaliplatin 85 mg/m² IV day 1
Leucovorin 400 mg/m² IV day 1
Fluorouracil 400 mg/m² IV bolus day 1
Fluorouracil 2400 mg/m² continuous IV infusion over 46 hours

An alternative CAPOX regimen every 21 days is:

Oxaliplatin 130 mg/m² IV day 1
Capecitabine 1000 mg/m² orally twice daily on days 1 to 14

Dose reduction is required for clinically significant renal impairment with capecitabine and for relevant treatment toxicity.

Metastatic disease

FOLFOX or CAPOX is a common first line approach for fit patients.

Test metastatic disease for mismatch repair deficiency or microsatellite instability. For MSI high or mismatch repair deficient advanced disease, immune checkpoint therapy is an important option.

A commonly used pembrolizumab regimen is:

Pembrolizumab 200 mg IV every 3 weeks or 400 mg IV every 6 weeks.

Obstruction from unresectable disease may require bypass or palliative resection when systemic therapy cannot restore gastrointestinal continuity quickly enough.

Hypersplenism

Overview

Clinical Features

Diagnosis

Management

Overview

Clinical Features

Diagnosis

Management