Non-neoplastic Lymphoid Proliferations.docx

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Biochem. and Medical Genetics
BGEN 3020
Jason Leboe- Mcgowan

Non-neoplastic Lymphoid Proliferations: Neutrophils – when you have acute inflammation = ie appendicitis, neutrophilic leukocytosis, left shift, toxic granulation, and leukamoid rxn. Leukamoid rxn means that it looks like leukemia but it isn’t and it’s benign. Usually involves any of cell lines. What causes leukamoid rxns? TB and sepsis. You see greater than 30-50,000 cells in the blood. Kids get these a lot (ie otitis media). Adult with otitis med = 12,000; kids with 30,000 (exaggerated). Example: Pertussus – whooping cough – lymphocytosis (60,000) – pediatricians are worried about ALL leukemia, but kid doesn’t have anemia or thrombocytopenia; kid comes in pale, coughing. Lymphocytes are mature and are totally normal. Lymphocytosis w/ viral infection or with pertussus. In atypical lymphocytosis – this is a lymphocyte that is doing what it’s supposed to do when presented to and Ag. It’s responding to the Ag by dividing and getting bigger, so basically it’s an antigenic stimulated lymphocyte. When talking about atypical lymphocyte, the absolute first thing that pops into the mind is: mononucleolosis – EBV. Other dz that are seen with large, beautifully staining bluish cells: CMV, toxoplasmosis, any cause of viral hepatitis, phenytoin. EBV is called the kissing dz b/c the virus holds up in the salivary glands. EBV affects B cells and CD 21. Mono causes viremia, generalized painful lymphadenopathy, very commonly get exudative tonsillitis, jaundice (hardly ever seen), increased transaminases (off the chart), and spleen enlargement and can rupture. Therefore don’t play sports b/c can ruptured spleen can occur, so avoid contact sports usually for 6-8 weeks. Also causes macrocytic anemia via inhibiting intestinal conjugase). Audio Day 3: Hematology File 5 Example: the boards will give you a classic hx of mono, and ask which tests you run, but monospot test is not on the choices b/c that’s the trade name, so pick heterophile antibodies (hetero = diff, phile = loving). Heterophile Ab’s are anti-horse RBC Ab’s (or anti-sheep); they are different, hence “hetero”phile Ab’s. Once you have mono, you always have it and will have 3-4 recurrences over your lifetime – ie reactivation consists of swollen glands, very tired, etc. EBV lives in B cells; the atypical lymphs in mono are T cells reacting against the infected B cells. Monocyte = king of chronic inflammation, therefore expect monocytosis in pts with chronic infections – ie rheumatoid arthritis, Crohn’s, ulcerative colitis, lupus, malignancy creatine gives energy b/c it binds to phosphate, and that is the phosphate you get from making ATP – so what serum test is markedly elevated in someone taking creatine for their muscles? Creatinine! B/c the end product of creatine metabolism is Creatinine. The BUN is normal in this person. Worthy board question. Eosinophilia You would see eosinophilia in Hay fever, rash in pt with PCN, strongoloides Protozoa infections DOES NOT produce eosinophilia, therefore it rules out amabiasis (pinworm), giardia, and malaria. Only invasive helminthes produces eosinophilia. Adult ascariasis does NOT cause eosinophilia b/c all they do is obstruct bowels, it’s when the invasive larvae form crosses into the lungs that causes eosinophilia. So anything that is Type I HPY causes eosinophilia; protozoa do not cause eosinophilia; ascariasis, and pinworms do NOT cause eosinophilia (all others – ie whipworms do b/c they invade). Myeloproliferative Dz: Polycythemia – increased RBC ct, increased Hb and Hct Difference between serum Na and total body Na? yes. Serum Na is milliequavalents per liter of plasma; total body Na is milliliters per kg body wt (the total amount you have). Similarly: RBC mass = total # of RBC’s in entire body in mL/kg in body wt RBC ct = # of RBC’s/microliter of blood, therefore its how many you have in a certain volume of blood. Why is this a big deal? Example: went running and vol depleted – RBC ct would be hemoconcentrated, therefore would look like more RBC’s per microliter of blood (b/c you depleted the plasma volume), but what would the RBC mass be? Normal (not actually synthesizing RBC’s). So, there are 2 types of RBC’s: relative and absolute. Relative = decrease in plasma vol causing an increase in RBC ct, but the RBC mass is normal. Absolute increase – is appropriate or inappropriate? When would it be appropriate? Syn of RBC’s – tissue hypoxia, so, any source of tissue hypoxia would be an appropriate response. Example: if you have lung dz, hypoxemia, COPD, high altitude – these are ie’s of appropriate polycythemias. What if we have normal blood gases, but didn’t have tissue hypoxia? This would be an inappropriate polycythemia. So, there are two things to think about with increased RBC mass: polycythemia rubivera, which is an ie of a stem cell proliferative dz of the BM, meaning that the stem cells are dictators, and nothing keeps them in check – a neoplastic dz; they can become leukemias. So, it would be inappropriate to have normal blood gases and no evidence of tissue hypoxia and have an increase in RBC mass. 2) Tumor or cyst with an excess production of EPO: renal adenocarcinoma making EPO, causing an increase in RBC mass – this is inappropriate b/c a tumor is inappropriately making it. In summary: polycythemia is relative or absolute. Relative means that you just lost plasma vol (ie from running) with RBC ct increased, and mass is normal. Absolute increase: is it appropriate or inappropriate? Appropriate – anything that is a hypoxic stimulus for EPO release. If there isn’t a hypoxic condition causing the EPO production, then you are ectopically making EPO from a tumor or cyst or you have polycythemia rubivera (a myeloproliferative dz). Intrinsic vs. Extrinsic Hemolytic anemia: 1. Intrinsic – something wrong with RBC, causing it to hemolyze: such as no spectrin, or not decay accelerating factor to neutralize complement, no G6PD enzyme in pentose phosphate shunt, or abnormal Hb (ie HbS). Therefore, something wrong inside the Hb molecule, causing it to hemolyze. 2. Extrinsic – nothing wrong with the RBC, just at the wrong place at the wrong time; ie it just happened to smash into the calcified valve (nothing was wrong with it, until it hit the valve). Then it will be dreading going to the cords of bilroth with destroy it b/c it has been marked with IgG and C3b for phagocytosis. Something intrinsically wrong with the RBC causing it to hemolyze but there’s nothing wrong with the BM (but something intrinsically wrong with the RBC), and the corrective ret ct is greater than 3%. MAD – MC intrinsic probs Membrane defect (spherocytosis, paroxysmal nocturnal hemoglobinuria), Abnormal Hb (SC trait Dz), Deficiency of enzyme (G6PD def). Membrane Defects: (a) Spherocytosis: do no see a central area of pallor therefore must be a spherocyte and must be removed extravascularly. Clinically manifest with jaundice from unconjugated bilirubin. Spectrin defect and AD dz; splenomegaly always seen over a period of time. Gallbladder (GB) dz is common b/c there is a lot more unconjugated bilirubin presented to the liver and more conjugation is occurring and more bilirubin is in the bile than usual. So, whenever you supersaturate anything that is a liquid, you run the risk of forming a stone; if you supersaturate urine with Ca, you run the risk of getting a Ca stone; if you supersaturate bile with cholesterol, you will get a cholesterol stone; if you supersaturate with bilirubin, you will get a Ca-bilirubinate stone. Therefore, pts have GB dz related to gallstone dz and then do a CBC with normocytic anemia and a corrected ret ct that is elevated, and see congenital spherocytosis. What’s the diagnostic test? Osmotic fragility – they put these RBC’s wall to wall in different tonicities of saline, and the RBC’s will pop (therefore have an increased osmotic fragility). Rx: splenectomy (need to remove organ that is removing them – they will still be spherocytes and will not be able to form a biconcave disk). (b) Paroxysmal Nocturnal Hemoglobinuria = defect in decay accelerating factor. So when we sleep, we have a mild resp acidosis b/c
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