Lymphadenopathy and Splenomegaly

Question 1

What is the history of splenomegaly?

Answer 1

Variable:
- Nil complaints
- Aware of mass
- Fullness and LOA
- Extreme pain (splenic infarct)
- Splenic rupture

Question 2

What is the differential for splenomegaly?

Answer 2

Infection
- Viral (EBV, CMV, HIV)
- Bacterial (IE, TB, brucellosis)
- Parasitic (malaria, bilharzia)

Infiltration
- Malignancy (myeloproliferative, lymphoproliferative)
- Amyloidosis
- Storage disease (Gaucher’s disease)

Inflammatory
- RA
- SLE
- Sarcoidosis

Congestive
- Cirrhosis
- Portal vein thrombosis

Haemolytic anaemia

Question 3

What are the causes of massive splenomegaly?

Answer 3

CML
Idiopathic myelofibrosis
Lymphoma
Hairy cell leukaemia
Gaucher
Thalassaemia
Malaria
Kala Azar (not in RSA)

Question 4

How does the FBC help in finding the cause of splenomegaly?

Answer 4

Normal -> bone marrow cause less likely, but can be lymphoma

Pancytopaenia -> hypersplenism or marrow involvement

Question 5

How does a blood smear help in finding the cause of splenomegaly?

Answer 5

Leucoerythroblastic picture: (infiltrationof bone marrow)
- nucleated RBC
- blasts
- tear drop cell

Question 6

What does the leukoerythroblastic picture look like?

Answer 6

Picture

Question 7

What does hairy cell leukaemia look like?

Answer 7

Picture

Indolent, chronic, low grade leukaemia

Question 8

What does CML look like?

Answer 8

Picture

Lots of neutrophils
Early granulocyte precursors

Question 9

What does CLL look like?

Answer 9

Picture

Small Lymphocytes

Question 10

When is splenectomy used?

Answer 10

Emergency:
- Traumatic rupture
- Spontaneous rupture

Therapeutic:
- ITP/ AIHA
- Hereditary spherocytosis
- Splenic marginal zone lymphoma
- Large painful spleen
- Hypersplenism

Diagnostic

High risk of thrombosis, infection, and life-threatening malaria/babesiosis

Question 11

What is the differentiation process of blood cells?

Answer 11

Picture

Question 12

What are the myeloproliferative neoplasms?

Answer 12

CML - Philadelphia chromosome t(9:22)

Ph- negative:
- Polycythemia Vera (incread Red cell production)
- Essential Thrombocythemia (increase platelet production)
- Primary myelofibrosis (increased fibroblast growth factor)

• can have JAK2V627F mutation
• splenomegaly and constitutional symptoms
• terminal phase pancytopenia and massive spleen
• can transform into AML

Question 13

What is the approach to lymphadenopathy?

Answer 13

Age
Onset
Size - increasing?
Systemic symptoms
Symptoms of infection/malignancy
HIV status
Medication

Question 14

What is the work up for lymphadenopathy?

Answer 14

FBC and smear
HIV
Tests for Systemic disease
Node biopsy

Question 15

How are lymphoproliferative disorders classified?

Answer 15

Leukaemia
Bone marrow origin
- Acute
- Chronic (CLL, HCL)

Lymphoma
Origin in nodes or spleen - bone marrow involved in advanced stage

Question 16

What are the types of lymphoma?

Answer 16

Hodgkin

Non-Hodgkin
- B cell
- Low grade (follicular)
- High grade (diffuse large cell, Burkitt, plasmablastic)
- T cell
- Low grade
- Aggressive

Question 17

What is CLL?

Answer 17

Slow growing, indolent

Commonest leukaemia

Night sweats, fatigue, anaemia, low platelets, lymphadenopathy, splenomegaly

Early finding may be incidental - might not require treatment (may not progress)

High risk of infection

Question 18

What is seen on node biopsy in Hodgkin lymphoma?

Answer 18

Picture

Question 19

What is Hodgkin lymphoma?

Answer 19

Slow growing - very good prognosis and cure rate in early stage
Can be aggressive in advanced stage

High incidence in advanced HIV

Managed with combination chemo and radiation to bulky nodal areas
Relapse can be cured with chemo and stem cell transplant if responsive

Question 20

How do lymph nodes biopsies work?

Answer 20

Should be given to everyone with unexplained lymphadenopathy
- Empiric TB therapy given to HIV + patients with lymphadenopathy

Fine-needle biopsy can diagnose TB or metastatic ca, but not lymphoma
Core biopsies can be done with biopsy gun
- can diagnose lymphoma
- can be excised if not diagnostic
- can diagnose

Biopsy the most representative node