Polycythemia (erythtrocytosis)

This is increase obove the normal range of RBCs in the circulacion. Concern that the

Hb level may be abnormally high should be triggered at a level of 170 g/L (17g/dL) in

men and 150 g/L (1g g/dL) in woman. Polycythemia found incidentally at routine

blood count. Relative erythrocytosis, due to plasma volume loss (e.g severe

dehidration, burns) does not represent a true increase in total RBC mass. Absolute

erythtrocytosis is a true in increase in total RBC mass.

Causes polycythemia vera (a clonal myeloproliferative disorder) erythtropoietin-

producing neoplasms ( e.g renal cancer, cerebellar hemangioma). Chronic hypoxemia

(e.g high altitude, pulmonary disease), carboxyhemoglobin excess (e.g smokers), high

affinity hemoglobin variants . cushing’s syndrome. Androgen excess. Polycytemia

vera is distinguished from secondary polycythemia by the presence of spenomegaly,

leukocitosis, trombocytosis and elevated vitamin B12 levels. An approach to evaluate

polycythemic pts is shown fig.

Complication hyperviscosity (with diminished o2 delivery) with risk of ischemic

organ injury and thrombosis (venous or arterial) are most common.

Treatment plebotomy recommended for hct ≥ 55%, regardless of cause to low normal

range.

Lymphadenopaty and splenomegaly

Exposure to antigen through a break in the skin or mucosa results in antigen being

taken up by an antigen – presenting cell and carried via lymphatic channels to the

nearest lymph node. Lymph channels course troughout the body except for the brain
and the bones. Lymph enters to the through the afferent vessel and leavels through an

effernt vessel. As antigen-presenting cells past through lymph nodes, they present

antigen-naive lymphocytes from the blood. They are retained in the node via special

homing receptors. B cells populate the the lymphoid follicles in the cortex ; T celss

populate the paracortical regions. When a b cell encounters an antigen to which its

surface immunoglobulin can bind, it stays in the follicle for a few days and froms a

germinal center where the immunoglobulin gene is mutated in an effoert to make an

antibody with higher affinity for the antigen. The b cell then migrates to the medullary

region, differentiates into plasma cell, and secretes imunoglobulin into the efferent

lymph. When a T cell in the node encounters an antigen in recognizes, it profiliferates

and joins the efferent lymph. The efferent lymph laden with antibodies and T cells

spesific for the inciting antigen passed through several nodes on its way tho the

thoracic duct, which drains lymph from most of the body. From the thoracic duct,

lymph enters the bloodstream at the left subcavian vein. Lymph from the head and

neck and the right arm drain into the raight subclavin vein. From the blood stream, the

antibodyvand T cells localize to the site of infection.

Lymphadenopathy may be caused by infections, immunologic diseases, malignancies, lipid

storage diseases, or a number of disorders of uncertain etiology (e.g.., sarcoidosis, Castleman’s

disease; Table 58-1). The two major mechanisms of lymphadenopathy are hyperplasia, in response

to immunologic or infectious stimuli, and infiltration, by cancer cells or lipid-or glycoprotein-

laden macro-phages.
 Approach to the Patient

History Age, occupation, animal exposures, sexual orientation, substance abuse history,

medication history, and concomitant symptoms influence diagnostic workup. Adenopathy is more

commonly malignant in origin in those over age 40. Farmers have an increased incidence of

brucellosis and lymphoma. Male homosexuals may have AIDS-associated adenopathy. Alcohol

and tobacco abuse increase risk of malignancy. Phenytoin may induce adenopathy. The

concomitant presence of cervical adenopathy with sore throat or with fever, night sweats, and

weight loss suggests particular diagnoses (mononucleosis in the former instance, Hodgkin's

disease in the latter).

Physical Examination Location of adenopathy, size, node texture, and the presence of

tendemess are important in differential diagnosis. Generalized adenopathy (three or more

anatomic regions) implies systemic infection or lymphoma. Subclavian or scalene adenOpathy is

always abnormal and should be biopsied. Nodes > 4 cm should be biopsied immediately. Rock

hard nodes fixed to surrounding soft tissue are usually a sign of metastatic carcinoma. Tender

nodes are most often benign. .

Laboratory Tests Usually lab tests are not required in the setting of localized adenopathy. lf

generalized adenopathy is noted, an exeisional node biopsy should be performed for diagnosis,

rather than a panoply of laboratory test.

TREATMENT

Pts over age 40, those with scalene or supraclavicular adenopathy, those with lymph nodes > 4 cm

in diameter, and those with hard nontender nodes should undergo immediate excisional biopsy. ln

younger patients with smaller nodes that are rubbery in consistency or tender, a period of

observation for 7-14 days is reasonable. Empirical antibiotics are not indicated. If the nodes shrink
no further evaluation is necessary. If they enlarge, excisional biopsy is indicated.

SPLENOMEGALY

Just as the lymph nodes are specialized to light pathogens in the tissues, the spleen is the lymphoid

organ specialized to fight bloodborne pathogens. lt has no afferent lymphatics. The spleen has

specialized areas like the lymph nodes for making antibodies (follicles) and amplifying antigen-

specific T cells (peiarteriolar lymphatic sheath, or PALS). In addition, it has a well-develop

reticuloendothelial system for removing particles and antibody-coated bacteria. The flow of blood

through the spleen permits it to filter pathogens from the blood and to maintain quality control

over erythrocytes (RBCs)-those that a cells specific for the inciting antigen passes through several

nodes on its way to the thoracic duct, which drains lymph from most of the body. From the

thoracic duct, lymph enters the bloodstream at the left subclavian vein. Lymph from the head and

neck and the right arm drain into the tight subclavian vein. From the bloodstream. the antibody

and T cells localize to the site of infection.