MASSIVE SPLENOMEGALY

Arphan Azaad

Chief Complain

Distention of abdomen

Left Upper Quadrant Pain(+)

x 2 months

Sweating+

Anorexia+

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

LUQ Tenderness+

ICTERIC(-)

ANEMIC(-)

Lymph Node(palpable nontender axillary,inguinal LN)

CLUBBING(-)

OEDEMA(-)

DEHYDRATION(-)

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 FUNCTION TEST:

Urea:8.9mmol/L,Cr:71umol/L

Na:141mmol/L,K:4.3mmol/L

LIVER FUNCTION TEST

TBI:18.1umol/L,DBI:6umol/L,TP:64.6g/L,AST:17U/L

SEROLOGY: HBeAg+, HBcAb+

ENDOSCOPY FINDINGS:

Chronic Gastritis ,Oesophagitis

CT FINDINGS

Massively enlarged and calcified Spleen

(159.6 mm x 79.3 mm)

Left Kidney cyst(14.2 x 6.4mm)

DIFFERENTIAL DIAGNOSIS

NON HODGKIN’S LYMPHOMA

HODGKIN’S LYMPHOMA

CHRONIC MYELOID LEUKEMIA

GAUCHER’S DISEASE

MALARIA,KALAZAR

MYELOFIBROSIS

LIVER CIRRHOSIS

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

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

INVESTIGATIONS REQUIREDDISEASE INVESTIGATION(GOLD

STANDARD)

HL & NHL LYMPH NODE BIOPSYIMMUNOPHENOTYPE

MYELOFIBROSIS BM STUDY

GAUCHER’S DISEASE MEASURE ACID BETA GLUCOSIDASE

MALARIA,KALAZAR BLOOD SMEAR,ISOLATION OF LD BODIES ON BM,SPLEEN SMEAR

CIRRHOSIS NODULAR DEGENERATION OF LIVER NOTED ON RADIO AND HISTOPATHOLOGICAL STUDY OF LIVER

CML BONE MARROW STUDY

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

Splenic Vein thrombosis should be considered.

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

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 Splenic Vein Thrombosis?

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

FOLLICUARLYMPHOMA

Germinal center B cell,CD10,BCL2&BCL6

T(14,18) involving BCL2 gene

GeneralizedLymphadenopathy Marrow involvement

MANTLE CELL LYMPHOMA

Naïve B Cell,CYCLIN D1 & usually CD5

T(11,14),INVOLVING BCL1( Cyclin D1 & Ig H )

Disseminated,moderately aggressive

DIAGNOSIS

MANTLE CELL LYMPHOMA IVB

Patient is currently being managed with supportive treatment to prevent Tumor Lysis Syndrome,managed with PPI & Prophylactic antibiotics.

PLAN:TO START RCHOP REGIMEN

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

TREATMENT

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

REFERENCE

Oncopedia-guidelines.info

Wikipedia:Mantle Cell Lymphoma

Harrison Principal of Medicine

Pathology Robbins

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