SPLENOMEGALY and LYMPHADENOPATHIES

1. SPLENOMEGALY and LYMPHADENOPATHIES Hasan Atilla Özkan, MD.

2. LYMPHADENOPATIES 1- Anatomy and Definitions • Lymph node regions in the body - head and neck - supraclavicular - deltopectoral - axillary - epitroclear - inguinal - popliteal

5. Normal lymph nodes are usually less than 1 cm in diameter (tend to be larger in adolescence than later in life) • Lymph nodes are often palpable in the inguinal region in healthy people, may also be papable in the neck (particularly submandibular) ; because chronic trauma and infection is more common in these regions

6. 2. Diagnostic approach A – History B – Physical examination C – Diagnostic tests

7. 1. History • Localizing signs or symptoms suggesting infection or malignancy • Exposures likely to be associated with infection (cat stratch disease, high risk behavior) • Constitutional symptoms such as fever, night sweets or weight loss • Use of medications that can cause lymphadenopathy • Foreign travel

10. 2. Physical examination All lymph node groups should be examined with the following characteristics in mind: • Location • Localized or generalized • Size • Tenderness • Consistency • Fixation

16. Location - 1 • Localized lymphadenopathy - suggest local causes, search for pathology in the area of node drainage - some systemic disease can also present with localized adenopathy * tularemia * aggressive lymphoma, etc

17. Location - 2 • Cervical adenopathy - bacterial infections - infectious mononucleosis - toxoplasmosis - tuberculosis - lymphoma - kikuchi’s disease - head and neck malignancies

18. Location – 3 • Supraclavicular lymphadenopathy - is associated with high risk of malignancy - right supra: mediastinum, lungs or esophagus - left supra (Virchow’s node): abdominal malignancy

19. Location - 4 • Axillary - drainage from the arm, thoracic wall and breast - infections are common causes -in the absence of upper extremity lesions, cancer is often found (particularly breast cancer)

20. Location - 5 • Epitrochlear - always pathologic - infections of the forearm and hand, lymphoma, sarcoidosis, tularemia and secondary syphilis • Inguinal - usually caused by lower extremity infection, sexually transmitted disease or cancer

21. Location - 6 • Generalized lymphadenopathy • Usually a manifestation of systemic disease * HIV infection * mycobacterial infection * infectious mononucleosis * systemic lupus erythematosis * medications * lymphoma / leukemia

22. 3. Diagnostic tests • Laboratory testing • CBC • Chest X-ray • PPD • HIV Ab • ANA • Other spesific test in need

23. Lymph node biopsy; (If an abnormal node has not resolved after 4 weeks or suspect of malignancy) - Open biopsy: genarally is the best test - Fine needle aspiration: useful when searching for reccurence of cancer - Core needle biopsy: in situtition where the open lymph node biopsy can not be performed

24. Incision and drainage • Imaging • Observation over time

25. SPLENOMEGALY • General Information • Hematopoietic organ capable of supporting elements of the erythroid, myeloid, megakaryositic, lymphoid and monocyte-macrophage systems • Participates in cellular and humoral immunity through its lymphoid elements • Removes senescent RBC, bacteria, and other particules from the circulation through monocyte-macrophage system (major function)

26. Splenectomized patients are suspectible to bacterial sepsis, especially with uncapsulated ones • Major lymphoid organ, containing ~ 25% of the total lymphoid mass of the body • About 1/3 of circulating plateletes are suspected in the spleen where they are in equilibrium with circulating plateletes

27. B. Size and Palpability • Median weight is about 150 grams • Average estimated weight of palpable spleen is about 285 grams • Not usually palpable, but may be felt in children, adolescents and some adults, especially those of asthenic build • A palpable spleen usually means the presence of significant splenomegaly • Enlarged spleen on physical examination is more reliable than minimally enlarged on imaging

28. The clincal or diagnostic significance of a spleen that is minimally enlarged on scan but is not palpable (scanomegaly) is uncertain • Symptoms of an enlarged spleen may include; - pain, a sense of fulness, or discomfprt in the left upper quadrant - pain referred to the left shoulder - early satiety, due to encrachment on the adjacent stomach

29. Criterias proposed to define the size of normal spleen; * USG – length < 13 cm or thichness ≤ 5 cm * CT scanning – length ≤ 10 cm

30. Causes of Splenomegaly • The causes of enlarged spleen are multiple: - most reflect the presence of hepatic or hematologic disease, infection or inflammation

31. Common causes - liver disease : 33% (cirrhosis) - hematologic malignancy : 27% (lymphoma) - Infection : 23% (AIDS, endocarditis) - congestion or inflammation : 8% - primary splenic disease: 4% (splenic vein thrombosis) - other or uncommon : 5%

33. Massive splenomegaly - chronic myeloid leukemia - myelofibrosis - gaucher disease - lymphoma - Kala-azar (visceral leishmaniasis) - malaria - beta-thalassemia major - AIDS with mycobacterium avium complex

36. D. Evaluation • History: - chronic alcholism, hepatitis - fatigue, fever, sore throat: inf. Mononucleosis - post-bath pruritis: polycytemia vera • Imaging studies • CBC and pheripheral blood smear • Bone marrow asp. and biopsy

37. Diagnostic splenctomy * most common pathologic diagnosis; - leukemia / lymphoma 57% - metastatic carcinoma 11% - cyst / pseudocyst 9% - beningn / malign vascular neoplasm 7% - normal 5% • Splenic aspiration / biopsy - is not widely practiced because of a concern for bleeding

38. General indications for splenectomy - isolated thrombocytopenia, hemolytic anemia or neutropenia - painfully enlarged spleen - traumatic or atraumatic splenic rupture - splenic artery aneurysm - hypersplenism - splenic vascular or parencymal lesion - to allow diagnosis

39. Spesific conditions in which splenectomy may be considered; • İmmune thrombocytopenia • Autoimmune hemolytic anemia • Thalassemia major or intermedia • Hereditory spherocytosis • Primary myelofibrosis • Hairy cell leukemia, splenic marginal zone lymphoma • Splenic contusion or rupture • Splenic abscess or infection • Splenic vein thrombosis with bleeding varices • Felty’s syndrome