SPLENOMEGALY : APPROACH

DR VASIF MAYANGMKMCH

INTRODUCTION Anatomy & Physiology

Classification

Etiology

Step-wise approach

Associated features

Investigations

ANATOMY• develops from condensations of mesoderm in the

dorsal mesogastrium.

• The weight of the normal adult spleen is 75–250 g.

• lies in the left hypochondrium between the gastric fundus and the left hemidiaphragm, with its long axis lying along the tenth rib.

• The hilum sits in the angle between the stomach and the kidney and is in contact with the tail of the pancreas.

The lower pole extends no further than the mid-axillary line. There is a notch on the inferolateral border, and this may be

palpated when the spleen is enlarged.

Normal spleenNormal size

12 cm length , 7 cm width13cm craniocaudal diameter

Weight < 250gmLocated along- 9th, 10th,11th ribs mid-axillarySpleen should be twice the size to be PALPABLEPalpable spleens are not always ABNORMAL3% normal population has palpable spleen

Splenomegaly : Spleen weight of 400-500gm 750 – 1000gm : prominent below costal margin >1000gm : massive splenomegaly

FUNCTIONSImmune function: The spleen processes foreign antigens major site of specific immunoglobulin M (IgM) production. The non-specific opsonins, properdin are synthesized. These antibodies are of B- and T-cell origin and bind to the

specific receptors on the surface of macrophages and leukocytes, stimulating their phagocytic, bactericidal and tumoricidal activity.

Filter function: Macrophages capture cellular and non-cellular material from

the blood and plasma. Remove old platelets and red blood cells. Iron is removed from the degraded hemoglobin during red

cell breakdown and is returned to the plasma. Removed non-cellular materials include bacteria particularly

capsulated organisms

Pitting: Particulate inclusions from red cells are removed and

repaired red cells are returned to the circulation. These include Howell–Jolly - nuclear remnants Heinz bodies - precipitated hemoglobin or globin subunits

EXAMINATION OF SPLEEN INSPECTION : Fullness in LUQ that descends on inspiration

Situated behind 9th, 10th and 11th ribs Long axis along line of 10th rib Anteriorly extends upto mid axillary line Posteriorly, the superior angle is 4cm lateral to D10 spine

PALPATION BIMANUAL PALPATION

BALLOTMENT

PALPATION FROM ABOVE - MIDDLETON MANOUVRE

BIMANUAL PALPATION 1. Place left palm firmly over left costal margin

posterolaterally and press it forward and medially. Palpate spleen with right hand starting from right iliac fossa

BIMANUAL PALPATION 2. Turn patient to right lateral position Palpate with right hand

MIDDLETON MANOUVRE Stand on left side Face foot end of patient Hooked fingers of LEFT HAND

placed under left costal margin

With right hand, pressure is exerted over posterolateral aspect of lower thorax

differences Sharp edge Notch –medial border Cross midline Moves with respiration Cannot get above it Neither Finger insinuation not possible Band of colonic resonance absent

Round edge No notch Not cross midline Donot moves with resp. Can get above it Bimanually palpable and ballotable Fingers can be insinuated Band of colonic resonance present

spleen kidney

PERCUSSION NIXONS METHOD Right lateral decubitus Start from posterior axillary line Upper border of dullness normally 6-8cm above costal margin >8cm dullness splenic enlargement

PERCUSSION CASTELLS METHOD Supine position Dull note over lowest ICS in Anterior axillary line on full

inspiration indicate splenomegaly

PERCUSSION TRAUBE’S SEMILUNAR SPACE left sixth rib superiorly left anterior axillary line

laterally left costal margin inferiorly

Dull note : splenomegaly

Differential diagnosis Enlarged left kidney Enlarged left lobe liver Carcinoma stomach Carcinoma splenic flexure colon Omental mass Malignancy tail of pancreas

splenomegaly

Massive beyond umblicus, crosses mid line >8cm below left costal margin and or >1000gm weight

Moderate b/w costal margin & umblicus (4-8cm)

Mild just palpable (1-3cm)

MECHANISM of SPLENOMEGALY 1. Hyperplasia or hypertrophy

Reticuloendothelial hyperplasia Immune hyperplasia in response to

A. systemic infectionB. immunological diseases

2. Passive congestion due to decreased blood flow

3. Infiltrative diseases

CAUSES OF SPLENOMEGALY Inflammatory splenomegaly Hyperplastic splenomegaly Infectious splenomegaly

Congestive splenomegaly Infiltrative splenomegaly

INCREASED DEMAND FORSPLENIC FUNCTION

1. INCREASED DEMAND A. RETICULOENDOTHELIAL SYSTEM HYPERPLASIA

SpherocytosisEarly sickle cell anemiaOvalocytosisThalassemia majorHemoglobinopathiesPNHPernicious anemia

INCREASED DEMAND B. IMMUNE HYPERPLASIA

(i) Response to infection IMN, viral hepatitis,AIDS,CMV,IE,TB, Histoplasmosis, Malaria,Bacterial sepsis,Cong syphilis, splenic abscess, Leishmaniasis, Trypanosmiasis, Ehrlichiosis

(ii) Disordered Immunoregulation RA (Felty syndrome), SLE, Collagen vascular diseases, AIHA,Immune thrombocytopenias, Drug reactions, Sarcoidosis, Interleukin2 therapy,Thyrotoxicosis

(iii) Extramedullary hematopoiesis : Myelofibrosis, Myelo infiltrative disorders, marrow damage by toxins,radiation.

2. Abnormal splenic/portal blood flow Cirrhosis Hepatic vein obstruction Portal vein obstruction Cavernous transformation of portal vein Splenic vein obstruction Splenic artery aneurysm Hepatic schistosomiasis CHF Hepatic echinococcosis Portal hypertension

3. Infiltration of Spleen Intracellular or

extracellular depositsAmyloidosisGaucher diseaseNiemann PickTangier diseaseHurlers syndrome, Other

MPSHyperlipidemias

Benign and Malignant InfiltrationsLeukemiasLymphomasHodgkins diseaseMyeloproliferative syndromesAngiosarcomasMetastasis ( MC= Melanoma)Eosinophilic GranulomaHistiocytosis XHamartomasHemangiomaSplenic cysts

4. Unknown Etiology Idiopathic splenomegaly Berylliosis Iron deficiency anemia

MASSIVE SPLENOMEGALY(>1000GM or >8cm) CML CLL Lymphoma Hairy cell leukemia Myelofibrosis with myeloid metaplasia Chronic malaria Kala - azar (Tropical Splenomegaly) Sarcoidosis Gaucher disease AIHA Diffuse splenic hemangiomatosis

Moderate splenomegaly(4-8cm) Viral hepatitis Cirrhosis Lymphomas Amyloidosis Splenic abscess,infarct Hemolytic anemias IMN Hemochromatosis Polycythemias

Mild splenomegaly (1-3cm) Acute malaria Typhoid kala-azar Septicemias SLE Infective endocarditis RA Thalassemia Minor Miliary TB Leptospirosis HIV CCF

Step-wise approach to splenomegaly

HistoryPhysical examinationLaboratory testsImagingSpecialised tests

history Mild vague, abdominal pain. Pain may be referred to the left shoulder. Early satiety from gastric displacement occurs with massive splenomegaly.

Associated symptoms or signs Febrile illness (infectious) Pallor, dyspnea, bruising, and/or petechiae (hemolytic process) History of liver disease (congestive) Weight loss, constitutional symptoms (neoplastic) Pancreatitis (splenic vein thrombosis) Alcoholism, hepatitis (cirrhosis)

Special situations associated with splenomegaly

Fever - typhoid,malaria,kalaazar, infect.endocarditis, leukemia,lymphoma Tender spleen - rupture,abscess,infarct a/c illness + anemia- AIHA, leukemia Fever + LN- IMN, leukemia, lymhomas, SLE, sarcoid Anemia- hemolytic anemia, hemoglobinopathies Jaundice – cirrhosis, hemolytic anemia Pulsatile spleen- aneurysm High ESR- connective tissue disorder Leukopenia- felty’s syndrome, septicemia

Physical examination Size of the spleen Hepatomegaly Lymphadenopathy Fever Icterus Bruising,petechiae Oral & supf.sepsis Stigmata of liver disease Stigmata of RA/SLE Splinter hemorrhage,retinal hemorrhage Cardiac murmurs

Lab investigations CBC Peripheral smear study Reticulocyte count Blood C/S Serology (viral, parasitic) LFT Hb electrophoresis / coombs test Coagulation profile Amylase/lipase AMA, Anti CCP, RA factor Bone marrow analysis

Imaging USG sensitive & specific non-invasive

CT scan - etiology of splenomegaly - liver size,heterogenecity - splenic mets, abscess,calcf.,cysts - retro peritoneal LN - cranio caudal ln > 12 cm

Liver- spleen colloid scan- (RBC – Cr51,Tc99) - hepatic steatosis, SOL, splenic functions - PHT, colloid shift +

MRI/ Doppler USG - portal / splenic vein thrombosis - cavernomas

imaging MRI scan liver hemangiomas hemochromatosis erlenmeyer flask sign (Gaucher)

PET scan Dx & staging of lymphomas determine metabolic cells in spleen

Specialised testing Abdominal fat pad aspiration JAK-2 mutation Gene testing ( bcr-abl , C282Y) Enzyme testing Lymph node biopsy FNAC spleen Splenectomy Liver biopsy