APPROACH TO SPLENOMEGALY
The spleen, an important organ in the reticuloendothelial system, plays key roles in red blood cell quality control, antibody synthesis, and removal of bacteria and red blood cells. Understanding normal spleen size and functions is crucial in diagnosing splenomegaly, which can be due to various causes such as immune hyperplasia, infections, and disorders of immunoregulation. This detailed guide by Dr. Maria George Jr. covers the anatomy, normal parameters, differential diagnosis, and implications of massive splenomegaly in clinical practice.
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APPROACH TO SPLENOMEGALY BY DR. MARIA GEORGE JR1 GENERAL MEDICINE
INTRODUCTION SPLEEN IS A RETICULOENDOTHELIAL ORGAN LOCATED IN THE LEFT UPPER QUADRANT ATTACHED TO THE STOMACH VIA THE GASTROLIENAL LIGAMENT AND KIDNEY VIA THE LIENORENAL LIGAMENT. ARISES AS A SERIES OF HILLOCKS IN THE DORSAL MESOGASTRIUM AT 5 WEEKS GESTATION AND MIGRATES TO NORMAL ADULT LOCATION. FAILURE OF FUSION OF HILLOCKS ACCESSORY SPLEENS.
NORMAL SPLEEN NORMAL SIZE 12CM LENGTH, 7 CM WIDTH (RADIONUCLIDE SCAN) - 13CM CRANIOCAUDAL DIAMETER ( USG ) WEIGHT < 250G LOCATED ALONG THE 9,10,11 RIBS MID AXILLARY LINE SPLEEN SHOULD BE ATLEAST TWICE THE SIZE TO BE PALPABLE. PALPABLE SPLEENS ARE NOT ALWAYS ABNORMAL NORMAL BLOOD FLOW TO SPLEEN 150ML/HR
MASSIVE SPLENOMEGALY PALPABLE > 8CM BELOW LEFT COSTAL MARGIN OR DRAINED WEIGHT >1000G
FUNCTIONS OF SPLEEN 1. QUALITY CONTROL OVER RBC REMOVAL OF SENESCENT AND DEFECTIVE RBC 2. SYNTHESIS OF ANTIBODIES 3. REMOVAL OF ANTIBODY COATED BACTERIA AND RBC
DIFFERENTIAL DIAGNOSIS OF SPLENOMEGALY 1. ENLARGEMENT DUE TO INCREASED DEMAND FOR SPLENIC FUNCTION RETICULOENDOTHELIAL SYSTEM HYPERPLASIA ( FOR REMOVAL OF DEFECTIVE RBC ) a) SPHEROCYTOSIS b) EARLY SICKLE CELL ANEMIA c) OVALOCYTOSIS d) THALASSEMIA MAJOR e) HEMOGLOBINOPATHIES f) PNH g) PERNICIOUS ANEMIA
2. IMMUNE HYPERPLASIA RESPONSE TO INFECTION a) INFECTIOUS MONONUCLEOSIS b) AIDS c) VIRAL HEPATITIS d) CYTOMEGALOVIRUS e) SUBACUTE BACTERIAL ENDOCARDITIS f) BACTERIAL SEPTICEMIA g) CONGENITAL SYPHILIS
h) SPLENIC ABSCESS i) TUBERCULOSIS J) HISTOPLASMOSIS k) MALARIA l) LEISHMANIASIS m) TRYPANOSOMIASIS n) EHRLICHIOSIS
DISORDERED IMMUNOREGULATION a) RHEUMATOID ARTHRITIS( FELTY S SYNDROME) g) DRUG REACTIONS h) ANGIOIMMUNOBLASTIC LYMPHADENOPATHY b) SYSTEMIC LUPUS ERYTHEMATOSIS c) COLLAGEN VASCULAR DISORDERS i)SARCOIDOSIS d) SERUM SICKNESS j) THYROTOXICOSIS e) IMMUNE THROMBOCYTPENIAS k) INTERLEUKIN 2 THERAPY f) IMMUNE NEUTROPENIAS
EXTRAMEDULLARY HEMATOPOEISIS a) MYELOFIBROSIS b) MARROW DAMAGE BY TOXIN, RADIATION, STRONTIUM c) MARROW INFILTRATION BY TUMORS, LEUKEMIAS, GAUCHER S DISEASE
2) ENLARGEMENT DUE TO ABNORMAL SPLENIC BLOOD FLOW 1. CIRRHOSIS 2. HEPATIC VEIN THROMBOSIS 3. PORTAL VEIN OBSTRUCTION ; INTRAHEPATIC OR EXTRAHEPATIC 4. CAVERNOUS TRANSFORMATION OF THE PORTAL VEIN 5. SPLENIC VEIN OBSTRUCTION 6. SPLENIC ARTERY ANEURYSM 7. HEPATIC SCHISTOSOMIASIS
8) CONGESTIVE HEART FAILURE 9) HEPATIC ECHINOCOCCOSIS 10) PORTAL HYPERTENSION
3) INFILTRATION OF THE SPLEEN INTRACELLULAR OR EXTRACELLULAR DEPOSITIONS; 1. AMYLOIDOSIS 2. GAUCHER S DISEASE 3. NIEMANN-PICK DISEASE 4. TANGIER DISEASE 5. HURLERS SYNDROME AND OTHER MUCOPOLYSACCHARIDOSES 6. HYPERLIPIDEMIAS
BENIGN AND MALIGNANT CELLULAR INFILTRATIONS 1. LEUKEMIAS( ACUTE, CHRONIC, LYMPHOID, MYELOID, MONOCYTIC) 2. LYMPHOMAS 3. HODGKIN S DISEASE 4. MYELOPROLIFERATIVE SYNDROMES ( EG : POLYCYTHEMIA VERA, ESSENTIAL THROMBOCYTOSIS) 5. ANGIOSARCOMAS
6. METASTATIC TUMORS 7. EOSINOPHILIC GRANULOMA 8. HISTIOCYTOSIS X 9. HAMARTOMAS 10. HEMANGIOMAS, FIBROMA, LYMPHANGIOMAS 11. SPLENIC CYSTS
UNKNOWN ETIOLOGY 1. IDIOPATHIC SPLENOMEGALY 2. BERYLLIOSIS 3. IRON DEFICIENCY ANEMIA
DISEASES ASSOCIATED WITH MASSIVE SPLENOMEGALY 1. CHRONIC MYELOID LEUKEMIA 6. GAUCHER S DISEASE 2. LYMPHOMAS 7. CHRONIC LYMPHOCYTIC LEUKEMIA 3. HAIRY CELL LEUKEMIA 8. SARCOIDOSIS 4. MYELOFIBROSIS WITH MYELOID METAPLASIA 9. AUTOIMMUNE HEMOLYTIC ANEMIA 10. DIFFUSE SPLENIC HEMANGIOMATOSIS 5. POLYCYTHEMIA VERA
APPROACH TO A PATIENT WITH SPLENOMEGALY CLINICAL ASSESSMENT SYMPTOMS: MILD SPLENOMEGALY MAY BE ASYMPTOMATIC MOST COMMON SYMPTOM : PAIN AND HEAVY SENSATION IN LEFT UPPER QUADRANT PAIN IS DUE TO ACUTE SWELLING OF SPLEEN LEADING TO STRETCHING OF CAPSULE/INFARCTION/INFLAMMATION OF CAPSULE MASSIVE SPLENOMEGALY EARLY SATIETY RUPTURE OF THE SPLEEN BREAKING THE CAPSULE IS ASSOCIATED WITH INTRA PERITONEAL BLEEDING, SHOCK AND DEATH. RUPTURE ITSELF MAYBE PAINLESS
PHARYNGITIS- INFECTIOUS MONONUCLEOSIS B SYMPTOMS OR WEIGHT LOSS -MALIGNANCY ( LYMPHOMA, MYELOPROLIFERATIVE NEOPLASM, METASTATIC SOLID TUMOR) ALCOHOL INTAKE OR NONALCOHOLIC FATTY LIVER DISEASE (NAFLD) MAY HAVE HEPATIC FIBROSIS OR CIRRHOSIS
PHYSICAL EXAMINATION FEVER-INFECTION OR HEMATOLOGIC MALIGNANCY SPLENIC TENDERNESS-INFARCTION, RUPTURE, OR ACUTE INFECTION ASCITES OR PERIPHERAL EDEMA -SEVERE LIVER DISEASE MASSIVELY ENLARGED SPLEEN-MYELOPROLIFERATIVE NEOPLASMS, LYMPHOMA, TRANSFUSION- DEPENDENT THALASSEMIA, GAUCHER DISEASE, OR TROPICAL SPLENOMEGALY SYNDROME
INSPECTION: FULLNESS IN LEFT UPPER QUADRANT WHICH DESCENDS ON INSPIRATION PALPATION 1. BIMANUAL PALPATION 2. BALLOTMENT 3. MIDDLETON MANEUVOR ( PALPATION FROM ABOVE)
FOR BIMANUAL PALPATION, THE PATIENT IS SUPINE WITH FLEXED KNEES. THE EXAMINERS LEFT HAND IS PLACED ON THE LOWER RIB CAGE AND PULLS THE SKIN TOWARD THE COSTAL MARGIN, ALLOWING THE FINGERTIPS OF THE RIGHT HAND TO FEEL THE TIP OF THE SPLEEN AS IT DESCENDS WHILE THE PATIENT INSPIRES SLOWLY, SMOOTHLY, AND DEEPLY. PALPATION IS BEGUN WITH THE RIGHT HAND IN THE LEFT LOWER QUADRANT WITH GRADUAL MOVEMENT TOWARD THE LEFT COSTAL MARGIN, THEREBY IDENTIFYING THE LOWER EDGE OF A MASSIVELY ENLARGED SPLEEN. WHEN THE SPLEEN TIP IS FELT, THE FINDING IS RECORDED AS CENTIMETERS
PERCUSSION FOR SPLENIC DULLNESS THE PATIENT IS PLACED ON THE RIGHT SIDE SO THAT THE SPLEEN LIES ABOVE THE COLON AND STOMACH. PERCUSSION BEGINS AT THE LOWER LEVEL OF PULMONARY RESONANCE IN THE POSTERIOR AXILLARY LINE AND PROCEEDS DIAGONALLY ALONG A PERPENDICULAR LINE TOWARD THE LOWER MIDANTERIOR COSTAL MARGIN. THE UPPER BORDER OF DULLNESS IS NORMALLY 6 8 CM ABOVE THE COSTAL MARGIN. DULLNESS >8 CM IN AN ADULT IS PRESUMED TO INDICATE SPLENIC ENLARGEMENT
WITH THE PATIENT SUPINE, PERCUSSION IN THE LOWEST INTERCOSTAL SPACE IN THE ANTERIOR AXILLARY LINE (EIGHTH OR NINTH) PRODUCES A RESONANT NOTE IF THE SPLEEN IS NORMAL IN SIZE. THIS IS TRUE DURING EXPIRATION OR FULL INSPIRATION. A DULL PERCUSSION NOTE ON FULL INSPIRATION SUGGESTS SPLENOMEGALY
PERCUSSION OF TRAUBES SEMILUNAR SPACE: THE BORDERS OF TRAUBE S SPACE ARE THE SIXTH RIB SUPERIORLY, THE LEFT MIDAXILLARY LINE LATERALLY, AND THE LEFT COSTAL MARGIN INFERIORLY. THE PATIENT IS SUPINE WITH THE LEFT ARM SLIGHTLY ABDUCTED. DURING NORMAL BREATHING, THIS SPACE IS PERCUSSED FROM MEDIAL TO LATERAL MARGINS, YIELDING A NORMAL RESONANT SOUND. A DULL PERCUSSION NOTE SUGGESTS SPLENOMEGALY
CBC AND BLOOD SMEAR ABNORMAL WHITE BLOOD CELLS (WBCS)-LYMPHOPROLIFERATIVE OR MYELOPROLIFERATIVE DISORDERS CYTOPENIAS LIVER DISEASE WITH HYPERSPLENISM, AIHA, ITP, FELTY SYNDROME, OR CONGENITAL DISORDERS (EG, HEREDITARY HEMOLYTIC ANEMIAS) TEARDROP CELLS MYELOFIBROSIS OR THALASSEMIA SPHEROCYTES AIHA OR HEREDITARY SPHEROCYTOSIS PARASITIC ORGANISMS IN BLOOD SMEAR. EXAMPLES INCLUDE EHRLICHIA SPECIES IN WBCS; OR BARTONELLA, BABESIA , MALARIAL PARASITE
OTHER INVESTIGATIONS LIVER FUNCTION TESTS ARE OFTEN HELPFUL IN DETERMINING THE CONTRIBUTION OF LIVER DISEASE HIV TESTING - IF NO OTHER CAUSE APPARENT BLOOD CULTURES ARE OBTAINED IF INFECTION IS SUSPECTED CHEST RADIOGRAPHY MAY BE USEFUL, AS PLEURAL EFFUSION MAY ACCOMPANY A SPLENIC ABSCESS GAUCHER DISEASE IS IDENTIFIED OR EXCLUDED USING A GLUCOCEREBROSIDASE ASSAY
FURTHER TESTING IN INDIVIDUALS WITH ABNORMAL INITIAL EVALUATION INDIVIDUALS WITH SICKLE CELL DISEASE (SCD), SPLENIC SEQUESTRATION CRISIS IS A POTENTIAL CONCERN, ESPECIALLY IN THOSE WITH AN ACUTE DECLINE IN HEMOGLOBIN OR SIGNS OF HYPOVOLEMIA SUSPECTED MALIGNANCY OR INFILTRATIVE DISEASE INVOLVING THE SPLEEN- BONE MARROW ASPIRATE AND BIOPSY IS APPROPRIATE IN MOST CASES OF SUSPECTED HEMATOLOGIC MALIGNANCY ALTERNATIVES INCLUDE LYMPH NODE BIOPSY OR TESTING OF PERIPHERAL BLOOD USING FLOW CYTOMETRY ( LYMPHOID NEOPLASMS) OR GENETIC TESTING (MYELOPROLIFERATIVE NEOPLASMS)
RHEUMATOLOGIC CONDITIONS OR HEMOLYTIC ANEMIAS, LABORATORY TESTING FOR AUTOANTIBODIES OR ABNORMAL RBC PROTEINS ABNORMAL LIVER FUNCTION TESTS AND SUSPECTED LIVER DISEASE, ULTRASOUND IMAGING OR ULTRASOUND-BASED ELASTOGRAPHY MAY BE USED, WITH LIVER BIOPSY IN CASES IN WHICH A TISSUE DIAGNOSIS IS INDICATED NONE OF THE ADDITIONAL TESTING REVEALS A DIAGNOSIS, IMAGING WITH COMPUTED TOMOGRAPHY (CT) OF THE CHEST, ABDOMEN, AND PELVIS MAY BE PERFORMED TO IDENTIFY LYMPHADENOPATHY OR OTHER AREAS TO BIOPSY, AND BONE MARROW ASPIRATE AND BIOPSY MAY BE PERFORMED
POSSIBLE INDICATIONS FOR BIOPSY SPLENOMEGALY WITH LEUCOCYTOSIS, ABNORMAL LYMPHOCYTES, OR IMMATURE WBCS ON THE PERIPHERAL BLOOD SMEAR SPLENOMEGALY WITH THROMBOCYTOSIS OR ERYTHROCYTOSIS MANAGEMENT (SPLENOMEGALY) MANAGEMENT DEPENDS ON THE CAUSE OF SPLENIC ENLARGEMENT. TREATMENT OF THE UNDERLYING CONDITION MAY REDUCE SPLENIC SIZE AND LEAD TO SYMPTOMATIC IMPROVEMENT
SPORTS PARTICIPATION AND FALL RISK SPLENOMEGALY IN THE SETTING OF INFECTIOUS MONONUCLEOSIS CARRIES A SMALL BUT INCREASED RISK OF SPLENIC RUPTURE THAT IS POTENTIALLY LIFE THREATENING ALL ATHLETES SHOULD REFRAIN FROM SPORTS AND OTHER ACTIVITIES WITH A HIGH RISK OF SPLENIC INJURY ROLE OF SPLENECTOMY SPLENECTOMY IS RARELY PURSUED AS THE FIRST CHOICE FOR MANAGING SYMPTOMATIC SPLENOMEGALY IN INDIVIDUALS UNDERWENT SPLENECTOMY FOR UNEXPLAINED SPLENOMEGALY, HALF HAD A NEOPLASTIC DISEASE, WITH SPLENIC MARGINAL ZONE LYMPHOMA THE MOST COMMON MALIGNANCY
