Why is the spleen enlarged massively?

You are given the command to examine the abdomen. You find that the spleen is enlarged to just below the umbilicus. Your whole viva may be conducted on the enlarged spleen. The following information will help you in answering viva questions. Do you need to percuss over the spleen? Only if you are in doubt as to whether the mass is the spleen or the kidney. This may happen if the spleen is enlarging straight down instead of diagonally across the abdomen and is mobile so may be mistaken for a palpable, ballotable kidney. Normally the spleen is plastered down to the back of the abdomen by the peritoneal membrane. In some people the spleen may have a mesentery so it becomes mobile and even a normal spleen may be felt in the flank or in left iliac fossa. A mass that cannot definitely be identified as a spleen needs to be percussed over. A resonant note over a kidney mass occurs because of overlying colon with air in it.

The spleen is tender. Why? Think of a splenic infarct or an abscess in the spleen. When are you likely to find an abscess in the spleen? When there is a septic embolus from subacute bacterial endocarditis, brucellosis, HIV with Mycobacterium avium complex, other septic emboli specially IV drug abuse. Splenic abscesses are rare and are treated with antibiotics and splenectomy.

When are you going to encounter a splenic infarct? In malignancies with a hypercoagulable state, the antiphospholipid syndrome, sickle-cell disease, atheromatous disease, atrial fibrillation if emboli are being thrown into the circulation, bacterial endocarditis, primary or secondary myelofibrosis (secondary to polycythemia vera), splenic lymphoma, blunt trauma to the abdomen and rarely in infectious mononucleosis, Gaucher’s syndrome and torsion of the mesentery of a mobile spleen. The presentation is abdominal pain, tenderness of the spleen, fever, nausea, leucocytosis.

Why is the spleen enlarged? It may be infiltrated: lymphoid tissue infiltrates it in a lymphoma, multiple myeloma, extramedullary hematopoeisis as in beta thalassemia minor and myelofibrosis, red cells may engorge it in congenital spherocytosis, abnormal white cells in chronic myelocytic leukemia, amyloid in amyloidosis, lipids in Gaucher,s disease, granulomas in mycobacterial infection. there may be a splenic angiosarcoma.

A massive spleen is seen in: chronic myeloid leukemia, myelofibrosis, primary or secondary to polycythemia vera or essential thrombocytosis, Gaucher”s disease, lymphoma, usually indolent, including hairy cell leukemia, Kala-azar (visceral leishmaniasis, this is seen in Pakistan in the Northern areas such as Baltistan and in a pocket in central Punjab), hyperreactive malarial splenomegaly syndrome, also called tropical splenomegaly syndrome), beta thalassemia major or severe beta thalassemia intermedia, AIDS with Mycobacterium avium complex. Have a thorough knowledge of thalassemia in the adult, the bony abnormalities seen in thalasemia major in children, how to follow-up a patient on transfusion therapy for anemia specially iron overloading and the importance of total serum iron levels, transferin saturation levels and ferritin and how to use the information you get from each test. you should also have knowledge of Mycobacterium ovum and other non-tuberculosis mycobacteria,

Kala-azar is an infectious disease, the vector is the sandfly (phlebotomus) which is found in Baltistan and the places listed below. The causative agent is Leishmania donovoni. Case reports have come in from Multan. Two cases reported in 1903 from Karachi were women who had traveled to Bangladesh. A history of travel to Afghanistan, Baltistan, Khyber-pakhtoonkhwa, Bangladesh,  Central Asia, Rajasthan area of India or Nepal may help. So do not just say a history of travel but say where the patient may have travelled to. Mass migrations and refugee populations are susceptible. The patient has fever, night sweats, weight loss, increase in skin pigmentation. Read up the tests that need to be done.

Cysts and pseudocysts — A splenic cyst (or multiple cysts) may be noted as an incidental finding on conventional imaging techniques, or as a result of evaluation of a patient with left upper quadrant pain, left shoulder pain, abdominal enlargement, or splenomegaly. Cysts are rare: post-traumatic cysts/pseudocysts, including cystic splenosis, Hydatid (echinococcal) cysts, congenital cysts, epidermoid, mesothelial cysts, hemangioma, lymphangioma, polycystic kidney disease with splenic cysts, splenic peliosis, cystic metastasis to the spleen are very rare.

Symptoms of an enlarged spleen. A minimally enlarged spleen secondary to an acute viral infection may be quite tender, while a markedly enlarged spleen in one of the chronic myeloproliferative disorders (eg, chronic myeloid leukemia. non-Hodgkin’s lymphoma, hairy cell leukemia, polycythemia vera, primary myelofibrosis) may be totally asymptomatic unless there is an episode of splenic infarction.

When present, symptoms of an enlarged spleen may include one or all of the following: pain, a sense of fullness, or discomfort in the left upper quadrant, pain referred to the left shoulder, pleuritic pain, early satiety, due to encroachment on the adjacent stomach. Look up Traube’s semilunar space in imaging or plain abdominal X-rays.

Pattern recognition in splenomegaly.

  • Enlarged firm to hard spleen, shrunken liver (reduced scan <11cm, will change with height of the patient), with or without ascites, history of jaundice or previous GI bleed or esophageal banding or the patient is aware of being Hep B or C positive or is an alcoholic. Diagnose cirrhosis, not CLD, with portal hypertension. Chronic liver disease spans a spectrum of liver disease from acute hepatitis, chronic active hepatitis, ( liver will be enlarged, ascites is unlikely, there may have been an episode of encephalopathy) early fibrosis, compensated cirrhosis, decompensated cirrhosis with portal hypertension. Which one does the patient you are examining have? Remember this is a practical examination, the viva is relevant to the current case and the management algorithm will change depending on which stage the patient is in.
  • Enlarged spleen with jaundice and anemia (the patient may even tell you that they have needed blood transfusions for years); think of a haemolytic anemia. The ones most likely are beta thalassemia major or intermedia, hereditary sperocytosis, other hemoglobinopathies such as hemoglobin D-Punjab, Hb E. Sickle cell disease is uncommon in Pakistan,
  • An older adult with a ruddy face and pruritus after bathing with hot water may have polycythemia rubra.
  • An adult with weight loss, anemia, recurrent episodes of infection and a massive spleen; think of chronic myeloid leukemia or myelofibrosis,
  • An enlarged spleen may be associated with arthritis or arthralgia as in Felty’s syndrome, SLE, rheumatoid arthritis and autoimmune haemolytic anemia.

Imaging of the spleen. 

  • ultrasound abdomen; >13 cm in length and 3.5 cm in thickness.
  • Plain X-ray of the abdomen may show an enlarged spleen
  • CT scan or PET scan.
  • Liver-spleen colloid scan.

Some important information.

  • the spleen may have a mesentery and may be mobile and may even wander down to the pelvis. Occasionally an IVU is needed to distinguish the spleen from the kidney.
  • an enlarged spleen is palpable not only because of its size but also because of its altered texture.
  • a biopsy of the spleen is never done; it is too vascular an organ.
  • a splenic ablation may be done by injecting sclerosants into the splenic artery.
  • portal hypertension can occur without cirrhosis of the liver i.e. portal vein thrombosis.
  • Remember NASH (non-alcoholic steato-hepatitis) and NAFLD (non-alcoholic fatty liver disease can cause portal hypertension and cirrhosis.
  • Hemoglobin S (can occur with thalassemia, hemoglobin C disease)  is seen in the Middle East, west coast of Africa and the Mediterranean countries like Italy, Greece, Spain, Lebanon. In Pakistan the Makran coast ancestry may give sickle-cell anemia.

 

 

Published by

shaheenmoin

I am a Professor of Medicine and a Nephrologist. Having served in the Army Medical College, Pakistan Army for 27 years I eventually became the Dean and Principal of the Bahria University Medical and Dental College Karachi from where I retired in 2016. My passion is teaching and mentoring young doctors. I am associated with the College of Physicians and Surgeons Pakistan as a Fellow and an examiner. I find that many young doctors make mistakes because they do not understand how they should answer questions; basically they do not understand why a question is being asked. My aim is to help them process the information they acquire as part of their education to answer questions, pass examinations and to best take care of patients without supervision of a consultant. Read my blog, interact and ask questions so that I can help you more.

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