- The patient has a clinical history suggestive of non-Hodgkin's lymphoma (NHL) including splenomegaly and lymphadenopathy. However, other diseases need to be ruled out including myelofibrosis, Gaucher's disease, and cirrhosis. - Laboratory tests found anemia and thrombocytopenia. Bone marrow biopsy did not find evidence of malignancy. - CT scan showed a massively enlarged and calcified spleen but no evidence of cirrhosis. - The diagnosis is mantle cell lymphoma stage IVB, an aggressive subtype of NHL. The patient will begin RCHOP chemotherapy regimen.

1. MASSIVE SPLENOMEGALY
Arphan Azaad

2. Chief Complain
Distention of abdomen
Left Upper Quadrant Pain(+)
x 2 months
Sweating+
Anorexia+

3. On Examination
General Condition :Fair
Vitals:
Chest=B/L clear BP=100/60mmHg
HR=88/min
T= 36.6C
R/R=20/min
CVS=S1.S2.M0
P/A= Soft Massive Splenomegaly
LUQTenderness+

4. ICTERIC(-)
ANEMIC(-)
Lymph Node(palpable nontender
axillary,inguinal LN)
CLUBBING(-)
OEDEMA(-)
DEHYDRATION(-)

5. INVESTIGATIONS
COMPLETE BLOOD COUNT:
WBC:16.1X10*9/L(N=20.7%),L(73.7%),
RBC:3.71X10*12/L,HB:107g/L,MCV:86.8fl
PLT:97X10*9/L, RET%:1.63%
RENAL FUNCTIONTEST:
Urea:8.9mmol/L,Cr:71umol/L
Na:141mmol/L,K:4.3mmol/L

6. LIVER FUNCTIONTEST
TBI:18.1umol/L,DBI:6umol/L,TP:64.6g/L,AST:17U/L
SEROLOGY: HBeAg+, HBcAb+
ENDOSCOPY FINDINGS:
Chronic Gastritis ,Oesophagitis

11. CT FINDINGS
Massively enlarged and calcified Spleen
(159.6 mm x 79.3 mm)
Left Kidney cyst(14.2 x 6.4mm)

12. DIFFERENTIAL DIAGNOSIS
NON HODGKIN’S LYMPHOMA
HODGKIN’S LYMPHOMA
CHRONIC MYELOID LEUKEMIA
GAUCHER’S DISEASE
MALARIA,KALAZAR
MYELOFIBROSIS
LIVER CIRRHOSIS

13. HL
• Suggesting :painless LN+,Splenomegaly
• Not suggesting:Cervical,Supraclavicular LN not
palpable,BM:absence of metastatic cells
NHL
• Suggesting:painless axillary,inguinal LN+,,Splenomegaly
• Not Suggesting : absence of Para Aortic
LN,palpable,BM:absence of metastatic cells
GAUCHER’S
DISEASE
• Suggesting:Massive Splenomegaly,Cytopenia
• Not Suggesting:more common in age group<20
years,absence of Pathological fractures

14. MALARIA
KALAZAR
• Suggesting:massive Splenomegaly
• Not suggesting:absence of fever virtually
ruling out any infection
MYELOFIBROSIS
• Suggesting:Massive Splenomegaly
• Non suggesting:absence of excessively
proliferating blood cells
CIRRHOSIS
• Suggesting:HBeAg+,HBcAb
• Non suggesting:absence of physical finding
and radiological findings of Cirrhosis

15. INVESTIGATIONS REQUIRED
DISEASE INVESTIGATION(GOLD
STANDARD)
HL & NHL LYMPH NODE BIOPSY
IMMUNOPHENOTYPE
MYELOFIBROSIS BM STUDY
GAUCHER’S DISEASE MEASURE ACID BETA
GLUCOSIDASE
MALARIA,KALAZAR BLOOD SMEAR,ISOLATION
OF LD BODIESON
BM,SPLEEN SMEAR
CIRRHOSIS NODULAR DEGENERATION
OF LIVER NOTED ON RADIO
AND HISTOPATHOLOGICAL
STUDY OF LIVER
CML BONE MARROW STUDY

16. SUMMARY
 Patient clinical h/o is highly suggestive of NHL.
 However we should also rule out other diseases
as I have mentioned on previous slides
 Elevation of CRP is highly suggestive of acute
infection ,but absence of fever does not suggest
it.
 BM does not suggest of malignancy
 SplenicVein thrombosis should be considered.

17. Absence of Hypersplenism
 Hypersplenism is most commonly seen with
splenomegaly due to hematologic
disorder,portal hypertension,Felty
Syndrome,Lymphoma.
 Hypersplenism produces:
Cytopenias
Normal or Hyperplastic bone marrow
Response to Splenectomy

18. Debatable Points
 Why not to do Splenectomy for symptomatic
relief of patient?
 Why to use IV antibiotics so early if infective
origin is least likely in the patient?
 Is it worthy to consider Lysosomal Storage
Disease and SplenicVeinThrombosis?

19. Neoplasm of Mature B Cells
NAME ORIGIN GENOTYPE FEATURES
BURKITT
LYMPHOMA
Germinal
center B
cell,CD10+
T(8,14).T(2,8) or
(8,22)
extranodal
abdominal masses
uncommonly
present as
Leukemia
DLBLC Germinal center
or post germinal
center B Cell
30% have
rearrangement of
BCL6,10% contain
translocation
t(14,18)
Rapidly growing
mass ,30%
extranodal
aggressive
FOLLICUAR
LYMPHOMA
Germinal center B
cell,CD10,BCL2&
BCL6
T(14,18) involving
BCL2 gene
Generalized
Lymphadenopathy
Marrow
involvement
MANTLE
CELL
LYMPHOMA
Naïve B
Cell,CYCLIN D1 &
usually CD5
T(11,14),INVOLVIN
G BCL1( Cyclin D1
& Ig H )
Disseminated,mod
erately aggressive

21. DIAGNOSIS
MANTLE CELL LYMPHOMA IVB
Patient is currently being managed with
supportive treatment to preventTumor Lysis
Syndrome,managed with PPI & Prophylactic
antibiotics.
PLAN:TO START RCHOP REGIMEN

23. MANTLE CELL LYMPHOMA
 Mantle cell lymphoma (MCL) is one of the rarest of the
non-Hodgkin's lymphomas(NHLs), comprising about 6% of
NHL cases.
 MCL is a subtype of B-cell lymphoma, due to CD5 positive
antigen-naive pregerminal center B-cell within the mantle
zone that surrounds normal germinal center follicles. MCL
cells generally over-express cyclin D1 due to a t(11:14)[2]
chromosomal translocation in the DNA. More specifically,
the translocation is at t(11;14)(q13;q32).[
 The immunophenotype profile consists of CD5+ (in about
80%),[7] CD10-/+,It is usually CD5+ and CD10.[8] CD20+,
CD23-/+ (though plus in rare cases). Generally, cyclin D1 is
expressed but it may not be required

24. TREATMENT

26. ECOG SCORING
 0 – Asymptomatic (Fully active, able to carry on all predisease activities
without restriction)
 1 – Symptomatic but completely ambulatory (Restricted in physically
strenuous activity but ambulatory and able to carry out work of a light or
sedentary nature. For example, light housework, office work)
 2 – Symptomatic, <50% in bed during the day (Ambulatory and capable of all
self care but unable to carry out any work activities. Up and about more than
50% of waking hours)
 3 – Symptomatic, >50% in bed, but not bedbound (Capable of only limited
self-care, confined to bed or chair 50% or more of waking hours)
 4 – Bedbound (Completely disabled.Cannot carry on any self-care.Totally
confined to bed or chair)
 5 – Death

27. REFERENCE
Oncopedia-guidelines.info
 Wikipedia:Mantle Cell Lymphoma
Harrison Principal of Medicine
Pathology Robbins

28. THANK YOU