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TUBERCULOUS SPLENOMEGALY Downstate Medical Center Wassim Abi Jaoude, MD March 2011 www.downstatesurgery.org

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Page 1: TUBERCULOUS SPLENOMEGALY - SUNY … · TUBERCULOUS SPLENOMEGALY [1] ... A sono or CT-guided percutaneous drainage of associated collections greater than 5 cm may be added to the ATT

TUBERCULOUS SPLENOMEGALYDownstate Medical CenterWassim Abi Jaoude, MDMarch 2011

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CASE PRESENTATION

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HPI 27 yo male presented with epistaxis x3 day

Admission 1/24/2011

Other associated symptoms Generalized weakness Anorexia Weight loss (50 lbs in 2 weeks) Night sweats

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PAST HISTORY Incarcerated for 10 years- released recently

Health deteriorated after release Esp. weakness and wt loss

Spontaneous pneumothorax 1-2 months prior to current admission Left thoracotomy with blebectomy Uneventful recovery

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PHYSICAL EXAM

VS

105/65

77/min

98F

General

Cachectic

Abdominal exam

Tender to deep

plapation

Hepatosplenomegaly

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ADMISSION LABS

CBC• WBC 4.6• Hb/Ht 9.8/31

LFTS• AST 59• AP 606• LDH 388

PLATELETS 8

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HEMATOLOGY Working Diagnosis

Idiopathic Thrombocytopenic Purpura

IVIG 0.5 g/kg daily x3daysCT Scan Abdomen/pelvis

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CT SCAN ABDOMEN AND PELVIS

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CT SCAN ABDOMEN AND PELVIS

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CT SCAN ABDOMEN AND PELVIS

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CT SCAN ABDOMEN AND PELVIS

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CT SCAN ABDOMEN AND PELVIS

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CT SCAN ABDOMEN AND PELVIS

Splenomegaly with diffuse heterogeneous enhancement and diffuse innumerable lesions

Soft tissue fullness in the porta hepatis and splenichilum- possibly LN

Hepatomegaly Retroperitoneal LN

R/O LYMPHOMA

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CT SCAN CHEST LLL 1.9 cm nodule with irregular margins

Bilateral hilar LN

Multiple enlarged mediastinal LN

R/O LYMPHOMA

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PET CT

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PET CT

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WORK UP/HOSPITAL COURSE

HD1PPD placed- negativeIVIG started at 25g/day x3days

HD3Evaluation by IR for possible CT-guided LN biopsyBone marrow biopsy done- megakaryocytic hyperplasia

HD4IVIG day 3- no major improvementDecadron started at 40 mg PO daily

HD5PLATELETS <5Platelet transfusion

HD6SURGERY CONSULT for evaluation for splenectomy

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WORK UP/HOSPITAL COURSE

HD6-7Transferred to surgery/monitored setting6 units platelets since admissionDDAVP

HD7Repeat CT A/P- evidence of gastric varicesand portal HTN

HD8

SPLENECTOMY- EBL 3000ml- 10 units PRBCs- 8 units single donor platelets- 2 units pooled donor platelets- 4 units FFP- 3000ml crystalloids

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OPERATIVE FINDINGS Large hepato-splenomegaly Well vascularized splenic ligaments Difficult dissection Intra-operative bleeding LUQ packing Abdomen kept open with VAC pack Transferred to ICU

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PATHOLOGY

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PATHOLOGY

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PATHOLOGY

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PATHOLOGY

• Entire spleen replaced by caseatinggranulomas

• Wt 1430 grams• 33x18x8cm • Granulomatous inflammation,

necrotizing type• Mycobacterium tuberculosis bacteria

present by AFB special stain• Hilar lymph nodes with non caseating

granulomas

SPLEEN

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POST-OPERATIVE COURSE

POD1 Started on anti-tuberculous medicationsAFB sputum negative x3

POD2OR for wash-outVAC pack reapplied

POD4OR for wash-outClosure with JP drain next to tail of pancreas

POD5-8Failed multiple attempts at extubationStarted on tube feedsFevers

POD10-11

ExtubatedTolerating diet

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POST-OPERATIVE COURSE

POD12 Blood cultures- Candida albicansStarted on Micafungin

POD13

Still spiking despite ATT and MicafunginRUQ sono- no cholecystitisRepeat CT AP

12 cm fluid collection in splenic lodgeSmall bilateral pleural effusions

POD14 IR Drainage of collectionSero-sanguinous- no improvement

POD15 & up

Patient still febrile, persistent fungemia, transferred out of ICU to the medical teamPlatelet count 870- ASA

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SPLENIC TUBERCULOSIS

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HITORICAL OVERVIEW PRIMARY TUBERCULOSIS OF THE SPLEEN-

coined by Coley in 1846 [1] Refers to an enlarged spleen from tuberculosis No or little involvement of other organs

1938- Englebreth-Holm changed it to TUBERCULOUS SPLENOMEGALY [1] Thought that the term Primary Tb of the Spleen is

misleading Considered that tuberculosis of the spleen must always

be secondary

Meredith HC et al

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GENERALITIES

• 15-20% of all cases of Tb [2]

ExtrapulmonaryTuberculosis

• 3-11% of extrapulmonary Tb [2,3]

Abdominal Tuberculosis

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FORMS AND INCIDENCE

SPLENIC TB OCCURS IN 2 FORMS

As part of MILIARY TBRelatively commonOccurs in immunocompromized patients

Spleen is the third organ involved (Lungs 100%, Liver 82%, Spleen 75%, LN 55%, Bone marrow 41%) [4]

Imani Fooladi AA et al

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FORMS AND INCIDENCE

ISOLATED splenic tuberculosisExtremely rare

Only 6 cases reported from 1965-1992 in the French, English and German literature [4]

Less than 100 cases reported in China [5]

Imani Foolady AA et al.Zhan et al.

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PREDISPOSING FACTORS [2,4,6]

Immunosuppression including AIDS Preceding pyogenic infections Splenic abnormalities Previous splenic trauma Sickle cell disease (and other hemopathies) Advanced age Diabetes Malnutrition In immunocompetent patients, there was usually

another site affected by Tb.Hamizah R et al; Imani Fooladi AA et al; Rhazal F et al.

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CLINICAL PRESENTATION

Fever [7] 82.3%

Fatigue and failure to thrive/weight loss [7]

44.12%

Splenomegaly [6,7] 13.2-100%

Asymptomatic leading to a delay in diagnosis up to 3 months [6]

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CLINICAL PRESENTATIONOther presentations

Pain- Uncommon [4]

Fulminant form- rapid progression with fever, cachexia, hemorrhage and sepsis [6]

Splenic rupture- rare [2]

Hypersplenism

Portal Hypertension- with or without GI bleeding [6]

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CLINICAL PRESENTATION/ HEMATOLOGIC ABNORMALITIES

TWO FORMS [8]

CYTOPENICMost common

Anemia- hemolytic, aplastic, megaloblastic…LeukopeniaThrombocytopenicPancytopenic

POLYCYTHEMIC ErythroblasticMyelocyticThrombocyticR/O myeloproliferative disorder

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DIAGNOSIS

PPDMost of the time positiveWeak argument in endemic countries [8]

PCR [4,8]Can identify the organism up to the speciesSerlogy or pathologic specimenSensitivity 53-93%Specificity 84-100%

Berady S et al; Imani Fooladi AA et al.

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DIAGNOSIS

SONOGRAM 5 pathomorphological types [5]

Miliary Tb Nodular Tb Tuberculous abscess Calcific Tb Mixed type Tb

TUBERCULOMAS [6,9] Multiple hypoechoic lesions, well demarcated w/o

posterior enhancement DD- Lymphoma, acute leukemia, angiomas,

metastases, fungal infectionsZhan et al; Rhazal et al; Sharma et al.

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DIAGNOSIS/SONOGRAM

TUBERCULOUS ABSCESS [6,9]

Hypoechoic or anechoic irregular lesions with posterior enhancement and sometimes containing debris

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DIAGNOSISCT SCAN [6,9] Tuberculomas

Hypodense, homogeneous, non enhancing, sometimes with a vascularized rim

Sensitivity in splenicabscess 90-100%

Differentiates MAC from M. tuberculosis (?)

Homogenous splenomegaly

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DIAGNOSIS

FNAC

Should be considered in front of any splenomegalyassociated with a fever of unknown origin [6[

Can involve the spleen, the liver or any accessible lymph node [5,6]

Can show granulomas with caseating necrosis [5,6]

Sensitivity 88%, specificity up to 100% [3,6]

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DIAGNOSIS

LAPAROSCOPY [5,10]

• Absence of interventional radiology capabilities• Failure of less invasive methods in establishing a

diagnosis

Indications

• Avoids unnecessary splenectomy• Provides direct hemostasis• Accurate

Advantages

Meshikhes et al

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TREATMENT

ANTI-TUBERCULOUS MEDICATIONS First line of treatment [2,5,6,7,8,11] Triple or quadruple therapy (INH, Rifampin, PZD…) Duration 12 months at least (up to 24 months in

one study) [2,5,11] Good response 86.6% [7]

A sono or CT-guided percutaneous drainage of associated collections greater than 5 cm may be added to the ATT [6]

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TREATMENT

SPLENECTOMY Not needed most of the time Still advocated by some authors to be an

adjunct to ATT [8,12]

DIAGNOSTIC [6] Recommended in case of failure of other

diagnostic methods

THERAPEUTIC

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TREATMENT/SPLENECTOMY

• Failure of medical treatment• In case of Cytopenia or

Polycythemia• Tuberculous splenomegaly with GI

bleeding due to PORTAL HTN• Failure of percutaneous drainage

of splenic abscess• Multiple abscesses of the spleen• PS- continue ATT after surgery

INDICATIONS [2,6]

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FOLLOW UP

MRI/PET If persistent lesions on US or CT scan Better suited to resolve stable fibrotic lesions

vs persistent active lesions PET is especially suitable for detecting the

activity of the lesions Crucial in assessing when to terminate

treatment [9]

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CONCLUSION Splenic Tuberculosis is a rare affection Most commonly associated with

immunocompromized states although it is also reported in immunocompetent patients

Most of the time there is another focus Attempts at diagnosis should be done to avoid

undue splenectomy First line therapy is medical Splenectomy reserved for diagnostic uncertainty

and cases refractory to medical treatment as well as in very specific conditions

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REFRENCES 1- Meredith HC, Early JQ, Becker W. Tuberculous

splenomegaly with the hypersplenism syndrome. Blood 1949; 4: 1367-73

2- Hamizah R, Rohana AG, Anwar SA et al. Splenictuberculosis presenting as pyrexia of unknown origin. Med J Malaysia 2007; 62(1): 70-71

3- Pottakkat B, Kumar A, Rastogi A et al. Tuberculosis of the spleen as a cause of fever of unknown origin and splenmegaly. Gut and Liver 2010; 4(1): 94-97

4- Imani Fooladi AA, Hosseini MJ, Azizi T. Splenictuberculosis: a case report. Int J Inf Dis 2009; 13: e273-e275

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REFERENCES 5- Zhan F, Wang CJ, Lin JZ et al. Isolated splenic

tuberculosis: a case report. World j GastrointestPathophysiol 2010; 1(3): 109-111

6- Rhazal F, Lahlou MK, Benamer S et al. Splenomegalie et pseudotumeur splenique d’originetuberculeuse: six nouvelles observations. Annales de Chirurgie 2004; 129: 410-414

7- Mazloom W, Marion A, Ferron C et al. Tuberculose splenique: a partir d’un cas et revue de la litterature. Medecine et Maladies Infectieuse 2002; 32: 444-446

8- Berady S, Rabhi M, Bahrouch L et al. La tuberculose splenique pseudotumorale. La Revue de Medecine Interne 2005; 26: 588-591

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REFERENCES 9- Sharma SK, Smith-Rohrberg D, Tahir M et al.

Radiological manifestations of splenic tuberculosis: a 23-patient case series from India. Indian J Med Res 2007; 125: 669-678

10- Meshikhes AWN, Al-Momen SAM. Laparoscopic diagnosis of splenic tuberculosis. Surg Laparosc EndoscPercutan Tech 2006; 16(5): 355-356

11- Ho PL, Chim CS, Yuen KY. Isolated splenictuberculosis presenting with pyrexia of unknown origin. Scand J Infect Dis 2000; 32: 700-701

12- Arab M, Mansoori D, Abbasidezfouli A et al. Splenic tuberculosis: a case report. Acta Medica Iranica2002; 40(1): 26-28

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