[Int. med] spleenomegaly from SIMS Lahore

A diagnostic approach to splenomegaly

Dr Nighat MajeedAssistant Professor Medical Unit IISIMS/SHL Lahore.

Anatomy

• It lies within the left upper quadrant of the peritoneal cavity.

• Abuts ribs 9-12, the stomach, the left kidney, the splenic flexure of the colon, and the tail of the pancreas.

Anatomy

http://www.oucom.ohiou.edu/tdi/topics_international_health/Images/fig52_splenomegaly.jpg

Anatomy• Normal Spleen

• Autopsy: <250g.

• Radioisotope Scintiscan: 12cm long x 7cm wide.

• Ultrasound: 11cm cephalocaudad diameter.

• ~3% of healthy people have splenomegaly.

Functions

• Immunosurveillance.• Hematopoiesis.• Clearance of microorganisms and particulate

antigens from the blood stream. • Synthesis of immunoglobulin G (IgG), properdin.

Tuftsin. • Removal of abnormal red blood cells.• Embryonic hematopoiesis in certain diseases.

Splenomegaly

Poulin et al defined splenomegaly on the basis of size of spleen

• Moderate; if the largest dimension is 11-20 cm.

• severe; if the largest dimension is greater than 20 cm.

Splenomegaly

Splenomegaly definition by weight• MILD; Spleens weighing 400-500 g.

Moderate; Spleen weighing 750-1000.

Massive; More than 1000 g to indicate massive splenomegaly.

Symptoms and signs

• Abdominal pain/tiredness.

• Cold/flu/Sore throat.• Early satiety due to splenic encroachment.• Symptoms of anemia due to accompanying

cytopenia. • Febrile illness (infectious). • Pallor, dyspnea, bruising, and/or petechiae

(hemolytic process).

Symptoms and signs

• History of liver disease (congestive).• • Weight loss, constitutional symptoms (neoplastic).• • Pancreatitis (splenic vein thrombosis).

• Alcoholism, hepatitis (cirrhosis).

Symptoms and signs

• Palpable left upper quadrant abdominal mass.

• Splenic rub.

• Lymphadenopathy.

Symptoms and signs

• Signs of cirrhosis (eg, asterixis, jaundice, telangiectasias, gynecomastia, caput medusa, ascites).

• Heart murmur (endocarditis, congestive failure). • Jaundice (spherocytosis, cirrhosis). • Petechiae (any cause of thrombocytopenia).

• Inspection

• Look in left

Hypochondrium.

Examination of the Spleen

Palpation of spleen

• To palpate the spleen, the patient is in the supine position with the knees flexed to decrease abdominal muscle tone.

• Begin the examination by palpating the right lower quadrant and move upward across the abdomen as the patient.

Palpation of spleen

Palpation• Right Lateral Decubitus.

Two-Handed.

Palpation of spleen

Supine Hooking Maneuver of Middleton”• Patient’s Fist under L CVA.• Stand on left facing patient’s feet.• Hook fingers over costal margin.

• A mass with notch in left upper quadrant indicate splenomegaly

Spleen vs. Kidney

Spleen• Splenic notch.• Can cross midline.• Can’t get above.• Moves down on

inspiration.• Not ballotable.• Splenic rub.

Kidney• No notch.• Never cross the

midline.• May get above.• Doesn’t move with

respiration.• Ballotable.• No rub.

Percussion of spleen

Normal • Left midaxillary line 9th –11th intercostal space

width 4-7cm.

• Enlargement of splenic dullness: splenomegaly.

Examination of the Spleen

Traube’s Space• Supine position.• 6th rib.• Costal margin.• Midaxillary line.• Normal breathing.• Splenomegaly = dullness

Percussion

Nixon’s Method

• Right Lateral.• Decubitus.• =8cm.

•

Percussion

• Castell’s Method• Supine p• Lowest intercostal

space• Left anterior axillary

line• Full inspiration and• expiration• Splenomegaly =

dullness

Palpation of spleen

• Percussion is also used to delineate the size of the spleen.

• Percussion is only approximately 60% accurate in most studies, with palpation about 50% accurate.

Principal causes of Splenomegaly

• Infection

1. Viral

2. Bacterial

3. Fungal

4. Rickettsial

5. Parasitic• Hemolytic anemia

• Cardiac failure • Trauma • Neoplasia • Portal hypertension • Metabolic disorders • Other


Infection • Bacterial (septicemia,endocarditic, cat scratch

disease, tularemia).• Brucellosis.• Tuberculosis.• splenic abscess. • Leptospirosis.• Lyme disease.• Syphilis.


Infection • In neonates,septicemia is most common. Usual pathogens are group B Streptococcus and E.

coli. • Enlarged spleen in infants, children, and

adolescents is due to acute viral infection,especially with Epstein-Barr virus or cytomegalovirus.


Infection • Fungal(histoplasmosis, candidiasis).• Rickettsial (Rocky Mountain spotted fever).• Parasitic (malaria, toxoplasmosis, visceral larva

migrans,schistosomiasis). • Kala-azar. • Viral hepatitis.


Haematological • Haemolytic anaemias (eg Thalassaemia, red cell

defects, Sickle cell anaemia). • Acute leukaemias, chronic leukaemias.• Polycythaemia rubra vera.• Macroglobulinaemia. • Lymphoma (Hodgkin's disease and non-Hodgkin's

lymphoma). • Essential thrombocythaemia. • Myelofibrosis.


Congestive splenomegaly

• Liver cirrhosis. • Budd Chiari syndrome. • Portal or splenic vein obstruction. • Heart failure.


Connective tissue disorders• Systemic lupus erythematosus.

• Felty's syndrome.• Connective tissue diseases (juvenile rheumatoid

arthritis, systemic vacuities).


Neoplasia Benign splenic tumors • Hemangioma.• Lymphangioma.• Hamartoma.

Malignancies • Acute lymphoblastic leukemia• Acute myeloid leukemia.• Hodgkin disease.• Non-Hodgkin lymphoma.


Portal Hypertension • Any cause of portal hypertension may cause

enlarged spleen. • Major causes are liver disease (cirrhosis, hepatitis,

extra hepatic biliary atresia);cavernous transformation of portal vessels; and portal or splenic vein thrombosis.


Metabolic Disorders • Amino acid disorders (tyrosinemia) • Carbohydrate disorders

(galactosemia,hereditary fructose intolerance) • Mucopolysaccharidoses (Hurler and Hunter

syndromes) • Lipidoses (Gaucher disease, Niemann-

Pickdisease, GM-1 gangliosidosis type I) • Glycoprotein disorders (sialidosis type II,

fucosidosis).


Metabolic Disorders • Histiocytosis X.

• Amyloidosis.


Other• Splenic cysts/Haemangiomas. • Inflammatory bowel disease. • Sarcoidosis. • Histiocytoses. • Drug hypersensitivity reactions.

Causes of massive splenomegaly

• Chronic myeloid leukaemia.

• Myelofibrosis, malaria (hyper-reactive malarial splenomegaly).

• Leishmaniasis.

• 'Tropical splenomegaly' (idiopathic; Africa, SE Asia).

• Gaucher's syndrome.

Splenomegaly in children

• Metastatic neuroblastoma.• Infection.• Autoimmune: juvenile rheumatoid arthritis.• Haemolysis: hereditary spherocytosis, sickle cell

anaemia, Thalassaemia • Neoplasia: ALL, Hodgkin disease and NHL, acute

or chronic myeloblastic leukemia, neuroblastoma. • Inherited diseases: Gaucher's disease and other

storage disorders.

Hypersplenism

Criteria for a diagnosis of hypersplenism anemia.

• Leukopenia.• Thrombocytopenia.• combinations thereof, plus cellular bone marrow,

splenomegaly, and improvement after splenectomy.

Approach to Splenomegaly

Depends on Pretest Probability• Clinical Suspicion of Splenomegaly (>10%).• Percuss first and if positive palpate.• If percussion is negative and suspicious, order an ultrasound.• If percussion positive but palpation is negative, order an ultrasound.• Both percussion and palpation positive = SPLENOMEGALY.

Diagnostic Approach

• CBC provides information about hematological, infectious, and inflammatory processes.

• Finding of pancytopenia, Anemia, Leukopenia,

Thrombocytopenia may indicate bone marrow dysfunction or portal hypertension with hypersplenism.

Laboratory tests

• Routine tests • CBC, platelet count, sedimentation rate.• chemistry panel, febrile agglutinins, serum

haptoglobins, ANA test, Monospot test, serum protein electrophoresis, tuberculin test.

• chest x-ray, EKG, and flat plate of the abdomen.

Diagnostic Approach

• Increased sedimentation rate suggests infectious, inflammatory, or neoplastic process.

• Bacterial, fungal, and other cultures may be performed with suspected infection.

Diagnostic Approach

• Bone marrow exam is useful in diagnosis of histiocytoses, lysosomal storage disorders, and some infections(e.g., disseminated histoplasmosis).

Diagnostic Approach

• Liver function tests and abdominalU/S with Doppler methods should be performed with suspected portal hypertension.

• • Abdominal U/S and CT locate and define extent of

splenic masses

If there is jaundice

• A hepatitis profile, red cell fragility test, and blood smear for parasites should be done.

If there is fever.• Serial blood cultures, leptospirosis antibody

titer, and smear for malarial parasites should be done.

Laboratory tests

Laboratory tests

If there is a petechial rash

• A coagulation profile should be done.

To rule out malignancies• Lymph node biopsies and bone marrow

examinations may be necessary.

Laboratory tests

• A CT scan of the abdomen and radio nuclide scan for liver and spleen size and ratio should be done.

• The assistance of a hematologist or infectious disease expert should be sought.

• A surgeon may need to be consulted for an exploratory laparotomy.

Imaging Studies

• Craniocaudal measurement: A craniocaudal measurement of 11-13 cm is frequently used as the upper limit of normal for splenic size in imaging studies.

• Computed tomography (CT) scanning

Imaging Studies

Splenoportography o This modality is used to evaluate portal vein

patency and the distribution of collateral vessels before shunt operations for cirrhosis.

o Findings can help identify the cause of idiopathic splenomegaly, especially in children.

• Angiography: Angiographic findings are used to differentiate splenic cysts from other splenic tumors.

Imaging Studies

• Liver-spleen colloid scanning o Erythrocytes are labeled with chromium-51 (51 Cr)

, mercury-197 (197 Hg), rubidium-81 (81 Rb), or technetium-99m (99m Tc), and the cells are altered by treatment with heat, antibody, chemicals, or metal ions so that the spleen sequesters them after infusion.

o A spleen length >14 cm is consider enlarged on liver-spleen scan

Procedures

• Splenectomy• Splenic biopsy• A liver biopsy,• splenic aspiration and biopsy.• Bone marrow biopsy may all be helpful in

diagnosing the reticuloendothelioses such as Gaucher's disease.

SPLEN O M EG ALY

AUTOIMMUNE DISORDER?AMYLOIDOSISCCF/SARCOIDOSIS

PORTAL HYPERTENSION

RBC find ing of haemolytic anemiasleucocytosis

ThrobocytosisPancytopenias

Abnormal

Absent

EBVToxoplasmosis

gauchers d isease

Approach to lym phadenopathyif present

lymphadenopathy

Normal

periphral blood filmT yp e tit le h e re

HISTORY ANDPHYSICAL EXAMINATION

Massive splenomegalyblood film

Bone marrow

SPLENOMEGALY

Observe

M ild asym ptom atic

lapro tom y

Negative

Bone m arrow biopsyCT abdom en

Explora tory laprotom y

Negative

Liver biopsy

Hepatom egaly with abnorm al liver functions

M oderate to m arked

lym phadenopathy absent nam e hereserolog ical studies negative

[Int. med] spleenomegaly from SIMS Lahore

Health & Medicine

Transcript of [Int. med] spleenomegaly from SIMS Lahore