PE - Approach to Splenomegaly

Question 1

List the most common causes of splenomegaly to appear in MRCP

Answer 1

1) Hematological malignancy
- divide into myeloproliferative and lymphoproliferative

2) Portal HT
- usually due to cirrhosis

3) Hemolytic anemia
4) Felty syndrome

Question 2

Outline the clinical features of myeloproliferative disease

Answer 2

1) No stigmata of chronic liver disease
2) Presence of conjunctival pallor
3) No scleral jaundice
4) No evidence of rheumatological disease
5) No lymphadenopathy
6) Excoriations marks suggest pruritus
7) Marked splenomegaly
8) No hepatomegaly, ascites, or peripheral edema
9) No hepatic venous hum
10) No signs of hepatic encephalopathy

anemia + pruritus + marked splenomegaly

Question 3

Outline the clinical features of lymphoproliferative disease

Answer 3

1) No stigmata of chronic liver disease
2) Presence of conjunctival pallor
3) No scleral jaundice
4) No evidence of rheumatological disease
5) Widespread lymphadenopathy
6) Excoriation marks suggest pruritus
7) Hepatosplenomegaly with mild splenomegaly
8) No ascites or peripheral edema
9) No hepatic venous hum
10) No signs of hepatic encephalopathy

anemia + pruritus + mild splenomegaly + lymphadenopathy + hepatomegaly

Question 4

Outline the clinical features of Felty syndrome

Answer 4

1) No stigmata of chronic liver disease
2) Presence of conjunctival pallor
3) No scleral jaundice
4) Symmetrical deforming arthropathy in small joints of hands + ulnar deviation + forearm nodules + vasculitic rash (?livedo reticularis)
5) Variable lymphadenopathy
6) No excoriation marks
7) Mild splenomegaly
8) No hepatomegaly, ascites, or peripheral edema
9) No hepatic venous hum
10) No signs of hepatic encephalopathy

anemia + mild splenomegaly + rheumatoid arthritis ± neutropenia (check CBC w/differential)

Question 5

What would be the most likely diagnosis in a patient w/scleral jaundice + no stigmata of chronic liver disease + splenomegaly?

Answer 5

Jaundice likely resulted from hemolysis in context of hematological malignancy

Cold autoimmune hemolytic anemia is especially associated w/chronic lymphocytic leukemia (CLL)

Question 6

In patients w/splenomegaly what could cause joint pain?

Answer 6

1) Hyperuricemia in context of hematological malignancy inducing acute gout
2) Felty syndrome which is a triad of seropositive RA + splenomegaly + neutropenia

Question 7

List all lymph nodes to check if patients present w/hepatosplenomegaly

Answer 7

1) Cervical
2) Supraclavicular
3) Infraclavicular
4) Axillary
5) Epitrochlear
6) Inguinal

Question 8

Outline the size of a normal and a palpable spleen

Answer 8

Normal spleen = around 12cm

Palpable spleen = more than 15cm

Question 9

Given that a mildly enlarged spleen will not be palpable what physical examination could screen for mild splenomegaly

Answer 9

Percussion over Traube’s space can detect subtle splenic enlargement; this space is normally occupied by the stomach so it is resonant while fasting; when the spleen enlarges this area becomes dull on percusion

Posterior border = left mid-axillary line/anterior axillary line
Superior border = left 6th rib
Inferior border = left costal margin

Question 10

Marked splenomegaly without lymphadenopathy

Answer 10

Myeloproliferative disorder

Question 11

Mild splenomegaly with lymphadenopathy ± hepatomegaly

Answer 11

Lymphoproliferative disorder

Question 12

List the 5 characteristics of a spleen on physical examination

Answer 12

1) Enlarges along Gardner’s line to the right iliac fossa
2) Unable to get over the mass
3) Dull on percussion
4) Not ballotable
5) Possesses a medial notch

Question 13

List the causes of splenomegaly by frequency

Answer 13

1) Portal HT
2) Hematological malignancy
3) Infection (e.g. endocarditis, HIV)
4) Congestion or inflammation (e.g. heart failure)
5) Primary splenic disease (e.g. splenic vein thrombosis)
6) Other

Question 14

List the causes of splenomegaly by spleen size

Answer 14

Massive splenomegaly = crosses midline

1) Chronic myeloid leukemia
2) Myelofibrosis
3) ADIS w/Mycobacterium avium complex
4) Malaria
5) Kala-azar (visceral leishmaniasis)

Moderate splenomegaly = does not cross midline

1) Portal HT
2) Acute/chronic leukemia
3) Lymphoma
4) Thalassemia
5) Glycogen storage disease

Mild splenomegaly

1) Hemolysis (e.g. hemolytic anemia)
2) Infection (e.g. EBV, infective endocarditis)
3) Autoimmune disease (e.g. RA, SLE)
4) Infiltrative conditions (e.g. amyloid, sarcoid)
5) Other myeloproliferative disease (e.g. polycythemia rubra vera)

Question 15

List conditions that cause isolated splenomegaly

Answer 15

1) Autoimmune diseases

2) Primary splenic diseases (e.g. splenic vein thrombosis, splenic abscess)

Question 16

Outline the B symptoms and explain its name

Answer 16

1) Fever >38 degrees
2) Weight loss >10% body weight in 6m
3) Night sweats

1/3 qualifies for B symptoms

In the Ann Arbor staging of lymphomas, A represents absence of systemic symptoms while B indicates their presence –> seen in malignancy, infection, or autoimmune conditions

Question 17

List important relevant history to ask in a patient w/splenomegaly

Answer 17

1) Risk factors for liver disease
2) Hx pancytopenia (bruising for PLT, fatigue and SOB for Hb, infections for WCC)
3) Risk factors for lymphoma (FHx, Hx RT, Hx chemotherapy, immunosuppressants, organ transplant)
4) HIV infection
5) Risk factors for autoimmune disease
6) Risk factors for malaria (travel Hx, prophylaxis)
7) Bone/joint paint (extramedullary hemopoiesis or secondary hyperuricemia)

Question 18

List investigations to perform in a patient w/splenomegaly

Answer 18

Blood tests

1) CBC w/differential for blasts
2) Blood film for blasts
3) RLFT
4) LDH elevated w/hemolysis, blast cells, lymphoma
5) Beta-2 microgloublin elevated w/multiple myeloma, lymphoma, and amyloidosis
6) Autoimmune profile
7) HIV antibody
8) BCR-ABL t(9;22) mRNA detection
- CML diagnosis + CML monitoring disease activity

Imaging

1) USG spleen
2) Axial CT chest, abdomen, pelvis for lymphadenopathy
3) PET for non-Hodgkin lymphoma

Biopsies

1) Lymph node biopsy
2) Bone marrow biopsy
- myeloid/lymphoid disorders
- lipid storage disease
- mycobacterial infection

Question 19

Outline the features of Felty syndrome

Answer 19

Triad of

1) Rheumatoid arthritis
2) Splenmegaly
3) Neutropenia

Question 20

Explain the clinical significance of blast crisis

Answer 20

1) Phase in hematological malignancy similar to acute leukemia
2) Characterized by presence of blast cells in
1. Bone marrow
2. Peripheral blood
3. Skin
4. Tissue

Question 21

List the causes of hyposplenism

Answer 21

1) Splenic infarction (e.g. sickle cell disease, vasculitis)
2) Splenic artery thrombosis
3) Infiltrative conditions (e.g. amyloid, sarcoid)
4) Celiac disease
5) Autoimmune disease

Question 22

Outline essential advice that must be given to patients w/hyposplenism or splenectomy

Answer 22

1) Vaccinations for encapsulated organisms w/repeat vaccinations every 5-10y –> Streptococccus pneumoniae, Haemophilus influenzae type B, Meningococcal C
2) Prophylactic antibiotics –> phenoxymethylpenicillin (penicillin V) or erythromycin for at least 2y
3) Attend AED immediately if patient becomes ill w/infection for administration of parenteral antibiotics
4) Carry an information card or wear a medic alert bracelet to inform healthcare providers of hyposplenism