Splenomegaly
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Transcript of Splenomegaly
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
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