Grey's presentation : Dr A Mahanga - Department of Health cystic. (1.2 - 1.9cm ) • Pancreas normal...
Transcript of Grey's presentation : Dr A Mahanga - Department of Health cystic. (1.2 - 1.9cm ) • Pancreas normal...
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GREYS PRESENTATION
Dr. A. Mahanga2006
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CASE HISTORY
31 year old Mrs B.M who presented with :• 2 week history of abdominal pain • Slow growing mass• 1 week history of epigastric pain, which
was non referred and worsening in intensity• No associated vomiting or nausea• No discoloration of eyes
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SYSTEMIC ENQUIRY
• Cough for 2/52• LOW• LOA• Nightsweats• Fatigue
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History...
• Pmhx : no D E A R T Hoff note , no previous TB+ TB contact
• Pshx : cholecystectomy - 1997
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ON EXAMINATION
• Stable but appears chronically ill looking• Alert and orientated• Vitals : Apyrexial BP = 100 / 70
P : 90 not distressed• No jaundice, pallor, clubbing, oedema• small cervical lymph nodes. Axillary LN <
1cm. No inguinal / epitrochlear
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ABDOMEN
• Distended• Fullness of epigastrium, RUQ• +/- 10 cm hepatomegaly, firm. Tender. No
nodules• 3 cm splenomegaly• Generalised abdo tenderness. - peritonitic• Bowel sounds present
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Systems
• Cardiovascular : JVP nS1 S2 normal. NIF
• Respiratory : Trachea centralPercussion - resonantGood air entry bilaterally
• Cns : Alert, orientatedNo meningism/focal sns
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Results - Blood
• FBC = 10.3 (94.8 / 29.9) , 7.21, 133• U+E = 130/3.5/104/23/4.0/75• LFT = 110/17/11/286/59/41• LDH = 1472• INR = 1.29
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Ultrasound Abdo• Liver is littered with hypodense lesions in
all segments. Non cystic. (1.2 - 1.9cm )• Pancreas normal• Spleen - Large hypodense mass (non cystic)
3.1 x 2 x 3.4 cm. Hypodense lesionin splenic hilum.
A) ? HEPATOMA = RECOMMEND CT
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CT ABDOMEN• Liver: A very large liver is seen. Poorly
defined masses seen throughout liver. No intra hepatic bile duct or vessel dilatation.
• Spleen: enlarged, with a large round hypodense lesion.
• Pancreas: normal• Kidneys: normal. Displaced inferiorly
RECOMMEND LIVER BIOPSY
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Liver biopsy
• Liver tissue represented: Infiltrated by Non - Hodgkins lymphoma high grade B cell lineage
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NONNON--HODGKINS HODGKINS LYMPHOMALYMPHOMA
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Background• Malignancies of lymphoid cells arise
from cells of the immune system at different stages of differentiation.
• Common haemopoetic progenitor = lymphoid < T cells and B cells.
• They may present as leukaemias(primary involvement of bone marrow and blood ) or lymphomas ( solid tumour of the immune system ).
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Epidemiology
• General decline in Hodgkins Disease rates in the past 20 years, making the disease only account for 1% of cancers.
• Non hodgkins however has increased by more than 70% in this same time.
• 54000 new cases of NHL diagnosed in USA in 2004.
• Age (40 - 70 ) > in elderly.
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• M > F ( higher rates in men in locations epidemic of HIV )
• > in whites than in blacks or asians.• 7th in frequency world-wide amongst all
cancers.• NHL is increasing in frequency,
worldwide.
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Pathophysiology
• NHL originate from lymphoid tissues (mainly lymph nodes, liver and spleen)
• The tumour cell line ie, B cell, T cell, hodgkins, correspond to the cellular components of antigen stimulated lymphoid tissue.
• There is clonal expansion of either B, T or natural killer cells.
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• 85 % of NHL are of B cell origin.• Only 15 % are derived from Tcell/ nkc• Some are derived from macrophages.• Classification : B cell - precursor
maturelarge BBurkitts
T cell - precursor mature
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Hodgkins - classicnodular
• The discovery of the Reed - Sternberg in the 20th century resulted in the distinguishing between HD and NHL.
• Furthermore the histological classification of NHL forms one of the most contentious issues in oncology
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Working formulationLow grade lymphoma• Peripheral adenopathy ( painless)• Spontaneous regression can occur,
confusing with a transient infection.• Extranodal and B symptoms -
uncommon.• Cytopaenia if bone marrow involved• Fatigue and weakness if advanced.
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Intermediate and high grade
• More varied clinical presentation.• Most patients have adenopathy• > 1/3 have extranodal involvement,
most commonly involving the GIT , skin, bone marrow, sinus, GUT, CNS.
• B symptoms more common (30-40%)
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GIT lymphoma
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• Primary CNS lymphomas are high grade neoplasms of B cell origin.
• Most CNS lymphomas are large cell / immunoblastomas.
• NHL accounts for 1 % of all CNS lymphomas
• These are commonly associated with immunosuppression.
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CNS lymphoma
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Who gets lymphomas?
• Immunodeficiency : HIVtransplantsinherited immune d.
• Environment : medical treatments, infectious diseasesagricultural chem.
• Hodgkins disease
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•Infections
• HTLV : infects T cells, T cell lymphoma• EBV : Burkitts lymphoma. Aggressive
NHL in immune suppressed. Majority of CNS lymphomas are associated with EBV.
• HIV• HELICOBACTER PYLORI : gastric malt• HEP C : lymphoplasmacytic lymphoma
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Burkitts in a child
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Genes
• A large number of translocations and mutations play a role in the differentiation of a tumour.
• In 30 % of pts with large B cell lymphoma there is a translocation t(14/18) -chromosome 18, which results in suppression of apoptosis( cell death ) and tumour formation.
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An approach to the patient
• Good history• Physical findings:
Peripheral lymph nodesSplenomegaly (40 %)HepatomegalyAbdominal / testicular massSkin lesions ( T cell lymphoma )
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Lab. studies
• FBC with diff count and platelets• Counts may be normal in early stage.• Anaemia, TCP, leucopaenia,
pancytopaenia may occur with bone marrow infiltration.
• Lymphocytosis• Thrombocytosis
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Chemistry
• Raised LDH ( poor prognostic factor )• Abnormal LFT ( hepatic involvement )• Hypercalcaemia ( acute form of T cell
lymphoma)• HIV• Raised B2 microglobulin• Monoclonal gammopathy
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Imaging
• CXR - 25% + nodes, effusion• Ultrasound abdomen• CT scan - neck, chest, abdo, pelvis • Bone scan - in bone pain / > ALP• Gallium scans• Upper GI series with follow through• MRI
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Procedures
• Biopsy - Lymph nodesFNA ( generally insufficient )heamotoxylin and eosin stain
• BMAT - not routine for diagnosis• Biopsy of extranodal sites• LP for CSF exam : in suspected primary
cns lymphoma, hiv, neuro abn.
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Staging- Ann arbor
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Differential diagnosis
• Infectious mononucleosis• Metastatic disease : carcinoma
melanomasarcoma
• Benign lymph node infiltrates, eg : TB, bacterial, fungal and viral infections
• Collagen vascular diseases
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HIV and lymphoma
• NHL most common lymphoma in HIV.• 2 types : a) systemic CD4 > 50
b) CNS CD4 < 50• The risk of developing systemic NHL is
60 - 200 x greater than general population
• Symptoms are commonly confused with most other infections common in HIV.
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HAART
• Although the incidence of various opportunistic infections as well as KS have fallen since the introduction of HAART, effects on NHL is unclear.
• Metanalysis of studies with 14936 HIV + pts showed that the relative risk of development of NHL in the era of HAART compared to pre-haart was statistically insignificant. (p = 0.58 )
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Treatment
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Treatment
• CHOP = combination chemotherapy • Cyclophosphamide, vincristine,
prednisone, doxorubicin.• Stage 1/2 : treated with 3- 4 cycles of
chemo then radiotherapy.• Cure rates are 80 - 90 % stage 1
60 - 70 % stage 2
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Treatment cont..
• Stage 2 (bulky), 3, 4 = 6-8 cycles of combination chemotherapy eg. CHOP often with Rituximab.
• If the pt responds initially, 70 % may achieve complete remission.
• 50 - 70 % may be cured• Those that do not respond require
salvage treatment.
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• NB: the long term disease free interval inpatients is < 10 %
• Studies have proved that pts with early stage NHL could be treated with a short course of chemo of lesser intensity, without irradiation of primary sites.(nejm oct 30 1997.)
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• Thomas. P. MiIller - investigated the use of chemo vs the use of chemo and radiotherapy in int./ high grade NHL
• This trial however showed that patients who received 3 cycles of CHOP and radiotherapy had better progression of disease and better overall survival than the use of chemo alone.(nejm July 1998)
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• Index case patient, sent to Greys.• She had routine Ct scans for staging• Sent to oncology, and later discharged for
palliative management
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Guess what he has??
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Thank you
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References
• Harrison’s: Principles of internal medicine• New England journal of medicine• Oncology channel• Research article, D.A Estrada and team,
haematology, oncology, cancer care specialists. E-medicine.