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53 year-old man (lunch vendor)

#Systemic Lymphadenopahy (submandibular , axillary , inguinal)

HPI

2weeks ago , he presented to our ER department with fever and headache. ER doctor suspected meningitis and took lumber puncture , and it showed pleocytosis (especially mononuclear cell) , elevation of CSF protein. The CSF culture and blood culture were negative .

Then he was diagnosed aseptic meningitis and hospitalized.

HPI

Admisson period : 7days #aciclovir 1,500mg/day 6days #ABCP 8g/day 5days #CTX 8g/day 5days ⇒Then fever and headache disappeared. BUT…. 　systemic lymphadenopathy persisted. Elevation of

implamation reaction(CRP) and hypoalbuminemia persisted.

So we followed him after discharge.

PMH,SH and FH

PMH:none

SH #smoking:1pack/day *30yeas #alcohol：some glass of beer 1time/month #occupation : lunch vendor #home : lives with wife,three children and his

mother #no pet

FH:unremarkable

PE

(vital sign) BP:110/70mmHg HR:80bpm RR:16bpm BT:36.8℃ (general) good (eye) not pale not icteric (oral) np (chest) no crackle no wheeze (heart) regular no murmur (abd) obesity no tenderness no hepatosplenomegaly

PE

(ext) no edema (skin) no rash (lymph node) no pain of ・1-2 palpable L/N of both sides of submandibular (both sides:2cm not so hard) ・1 palpable L/N of both sides of inguinal (right side:5cm left side:3cm hard elastic) (etc) no weight loss

Time course of labo data

Admission Follow 1 Follow 2 Follow 3 Follow 4 Follow 5 Follow 6

WBC 13,400 12,400 5,300 8,800 8,100 8,800 9,200RCB 433 427 453 461 441 435 429Hgb 13.0 12.4 13.2 13.5 12.9 13.0 12.9Hct 38.2 37.6 39.6 41.2 39.2 39.2 38.4MCV 88 88 87 89 89 90 90Plt 33.4 29.5 36.8 43.3 42.8 32.2 34.7Na 125 125 132 136 133 138 136K 4.8 4.2 4.5 4.8 4.2 4.0 3.7Ca - - 7.7 8.2 8.5 8.4 8.7

During hospitalization Outpatient department

Time course of labo data

Admission Follow 1 Follow 2 Follow 3 Follow 4 Follow 5 Follow 6

TP - 9.1 - - 10.1 10.4 -Alb - 2.4 - - 2.9 2.9 -BUN 11 11 8 8 13 11 8Cre 0.8 0.7 0.7 0.7 0.7 0.8 0.8AST 46 35 49 57 75 47 50ALT 13 12 24 37 63 21 20CRP 11.86 12.15 9.25 6.64 6.26 7.08 7.34ESR - - - - - 117.0 -

During hospitalization Outpatient department

Prolonged inflammation reaction on labo data

0

2

4

6

8

10

12

14

0

2,000

4,000

6,000

8,000

10,000

12,000

14,000

16,000

Admission Follow 1 Follow 2 Follow 3 Follow 4 Follow 5 Follow 6

WBC CRP

WBC CRP

★ 117

ESR

Outpatient departmentDuring hospitalization

CT scan

#lymphadenopathy :axillary(1cm), para aorta(5cm) ,para pancreas head(3cm),inguinal(5cm)

#No space-occupying lesion of lung, no hepatosplenomegaly

Problem List

# systemic lymphadenopathy # post aseptic meningitis # elevation of inflamation reaction (WBC,CRP,ESR)

on labo data without subjective symptom # hypoalbuminemia(low A/G)

Differential diagnosis

#Viral illness(ex.CMV EBV) #HIV #ATL #Lymphoma #Castleman disease #Sarcoidosis #TB #Toxoplasma

How to diagnose?

#Viral illness(ex.CMV EBV) :IgG,IgM antibody #HIV: HIV1/2 #ATL: HTLV-1 #Lymphoma :biopsy #Castleman disease: biopsy ,IL-6 #Sarcoidosis : ACE #TB: non-specific TB IFN-γ

Additional Exam 1 Electrophoresis

1.Alb:33.7%(↓) 2.α1-glb:2.2%(→) 3.α2-glb:7.4%(→) 4.β-glb:6.2%(↓) 5.γ-glb:50.5%(↑) A/G:0.5(↓)

#no BJP #no M-protein

Hyper γ-globulinemia with a polyclonal pattern

Additional Exam 2

HIV1/2:(-) HTLV-1:(-) CMV IgM(EIA):0.79(-) CMV IgG(EIA):30.6(+) EBV VCA IgM(EIA):0(-) EBV VCA IgG(EIA):9.3(+) EBV EBNA IgG(EIA):3.6(+) Toxoplasma IgG ab:(+) Toxoplasma IgM ab:(-)

ANA:×160 (< 40) IgG:5,046mg/dl (880-1,800) IgM:700mg/dl (52-270) IgA:464mg/dl (126-517) IgG4:176 (4.8-105) IL-2R:1,270 (145-519) IL-6:13.8 (< 4.0) ACE:13.2 (8.3-21.4) non-specific TB IFN-γ:(+)

Past infection

Past infection?

summary

53 year-old Japanese man post aseptic meningitis , he showed

1.Systemic lymphadenopathy 2.Elevated serum CRP , ESR levels 3.Hyper γ-globulinemia with a polyclonal pattern 4.Additional exam showed abnormal ( IgG,IgM,IL-6 etc)

Differential diagnosis

#Viral illness(ex.CMV EBV) #HIV #ATL #Lymphoma #Castleman disease #Sarcoidosis #TB #Toxoplasma

Biopsy

From right side of inguinal (5cm)

Biopsy (right inguinal lymphnode)

# IgG4 stain showed no findings

#Lymphnode biopsy showed infiltrate plasma cell around a lymph follicle

Definite Diagnosis

Multicentric Castleman disease plasma cell type

We plan to use prednisolone on department of hematology.