White Cell Disorders: Lymphoproliferative disorders Flashcards
what are the primary lymphoid organs?
thymus
bone marrow
what are the secondary lymphoid organs?
lymph nodes
spleen
mucosa associated lymphoid tissue
what is the role of the primary lymphoid organs?
Generate mature naïve lymphocytes from immature progenitor cells
what is the role of the secondary lymphoid organs?
Are the sites of lymphocyte activation by antigen. Activation leads to clonal expansion and affinity maturation. Mature lymphocytes recirculate between the blood and the peripheral lymphoid organs until they encounter their specific antigen.
causes of lymphocytosis
benign:
- viral: EBV, CMV, HIV, rubella, measles
- bacterial: pertussis, brucellosis, TB
- varies with age
- post splenectomy
- smoking
malignant:
-lymphoproliferative disorders: CLL, waldenstrom’s, mycosis fungoides
investigations for lymphocytosis
Full history and examination
Reactive/Viral
- Repeat in 6-12 weeks
- Consider assessing for underlying cause e.g. EBV
If persistently >5x109/L and no clear cause consider:
- Immunophenotyping on peripheral blood
- Staging Ix (CT, Bone marrow biopsy)
- Biopsy of target lesion (if present)
presentation of infectious mononucleosis
Benign, self-limiting Between the ages 10-25 yrs Fever, malaise, and sore throat Lymphadenopathy and splenomegaly Hepatomegaly and jaundice
lab investigations for infectious mononucleosis
Heterophile antibody test: Monospot Specific antibodies for EBV antigens: viral capsid antigen (VCA) early antigen (EA) EBV nuclear antigen (EBNA)
aetiology of lymphoproliferative disorders
viral- HIV, EBV, HHV8
bacteria: H. pylori, C jejuni
Parasites: malaria
AI
Immune deficiency
environmental e.g. radiation, chemical, drugs
staging investigations for lymphocytosis
History – symptoms suggesting advanced disease, previous immunosuppressive illness, previous malignancy and chemotherapy or radiotherapy
Examination for lymphadenopathy or organomegaly (including Waldeyer’s ring)
Haematology: FBC, blood film, ESR, blood group and screen, coagulation screen
Serum chemistry: renal and liver function, bone profile, LDH, urate, CRP, immunoglobulins, serum protein electrophoresis, β2 microglobulin
Virology: HbsAg, HbsAb, HbcAb, anti HCV, VZV IgG, CMV IgG
Biopsy
Bone marrow biopsy
Imaging – CXR, CT of thorax, abdomen and pelvis, FDG-PET (if indicated MRI, ECHO,MUGA)
Lumbar puncture in aggressive NHL
clinical features of lymphoma
Painless lymphadenopathy (HL contiguous & often localised in contrast to NHL) Constitutional (B) symptoms: fever 380C for 3 d) night sweats weight loss (> 10% BW) Pruritus Alcohol induced pain (rare)
Impaired immune response (humoral and cell mediated)
Hepatosplenomegaly
Occasionally skin , tonsils & adenoids, gastrointestinal tract, lungs, CNS, jaw, testis
lab features of lymphoma
Haematology normochromic, normocytic anaemia leucoerythroblastic blood film hypersplenism neutropenia and thrombocytopenia autoimmune cytopenias (DAT) circulating lymphoma cells (CLL, FL, MCL,DLBCL) raised ESR, CRP
biochemistry results of lymphoma
raised lactate dehydrogenase and hypoalbuminaemia Serum or urine paraproteins (15%) b 2-microglobin normal immunoglobulins reduced hypercalcaemia (ATLL, advanced dis) abnormalities of liver or renal function
symptoms of splenomegaly
No symptoms in some cases
Pain or fullness in the left upper abdomen that may spread to the left shoulder
Feeling full without eating or after eating only a small amount — this can occur when an enlarged spleen presses on the stomach
Anaemia
Fatigue
Frequent infections
Easy bleeding
causes of splenomegaly
CML CLL ALL thalassaemia major or minor haemolytic anaemia portal hypertension cirrhosis sarcoidosis infection e.g. sepsis, bacterial endocarditis, typhoid, TB, malaria
