Heme Ck

mikenakhla's version from 2016-05-21 15:14



Question Answer
• These are found in patients with splenectomies (typically sickle cell)howell jolly bodies
• How much hgb A do patients with sickle trait have?50-60 percent
• Basically the only manifestation of sickle traitrenal (painless hematuria, papillary necrosis, can't concentrate urine as well)
• Differentiate between iron deficiency and thalassemia using this lab valueRDW elevated with iron deficiency
• Hemolysis leading to hemoglobinuria, anemia (fatigue and dyspnea) and hypercoagulable state leading to acute abdominal pain eg portal vein thrombosis in 4th decade of lifeparoxysmal nocturnal hemoglobinuria due to CD55 and CD59 anchor protein on RBC defect
• High IgM but low IgG and IgA and recurrent sinus infections and sinusitisHyper IgM syndrome. Defect in CD40 igand. Normal lymphocyte count
• Low IgA, IgG, IgM and low or absent lymphocyte count? What about low IgA IgG IgM and normal lymphocyte count?bruton agammaglobulinemia, common variable immunodeficiency
• Adenosine deaminase deficiencyimpaired T cell development, recurrent infections. causes 15% of SCID cases
• Isolated low platelet count, what is this and how do you treat it in adults? In children?this is ITP. In adults if platelet count is > 30k and no bleeding, observe. If less than 30k or bleeding, IVIG or steroids. In children if they have purpura only, just observe. If they're bleeding, IVIG or steroids
• Hypercalcemia, anemia, renal failure. What is this and how do you diagnose it?multiple myeloma, do a serum electrophoresis looking for an M spike. Bence jones proteins are In the urine
• Radiation, drugs (chemo or abx), infection, or toxins leading to anemia, leukopenia, reticulocytopenia, and thrombocytopenia. What is this and how do you make the diagnosis?acquired aplastic anemia. Need bone marrow biopsy to make the diagnosis.
• Pancytopenia with hypopigmentation on the trunk, neck, possible café au lait spots, short, skeletal abnormalities, renal malformations, other thingsfanconi anemia.
• Absent CD3 (T Cells), low/dysfunctional CD19 (B cells). Recurrent infections, failure to thrive. What is this and how do you treat it long term?this is SCID, severe combined immunodeficiency. Only definitive treatment is a stem cell transplant
• Hemolytic episodes of G6PD can be precipitated byinfections or meds (sulfa, antimalarials, nitrofurantoin)
• Sickle cell patient with acute severe shortness of break, weakness, fatigue, no chest painaplastic crisis due to parvovirus B19 infection. Low retics and how hemoglobin
• Therapeutic INR for idiopathic thromboenbolism? What if you have a prosthetic heart valve?2-3, 2.5 to 3.5 with the valve
• X linked disorder, eczema, thrombocytopenia, hypogammaglobulinemiawiskott aldrich syndrome
• PAS+, TdT +ALL
• CF positive, which coagulation factors might be deficient?Vitamin K is not absorbed (b/c fat soluble) and it's important for the liver to make factors II, VII, IX, and X as well as proteins C and S
• Treatment of P veraphlebotomy to lower HCT
• Basophilic stipplinglead poisoning
• Howelll jolly bodiesasplenia
• Schistocytes, helmet cellsIntravascular hemolysis (microangiopathic hemolytic anemia and DIC)
• Acanthocytes, spur cellsabetalipoproteinemia
• Target cellshemoglobin C disease and liver disease
• RBC morphology harllmark of uremia?echinocytes aka burr cells
• Ringed sideroblastssideroblastic anemia
• Which lab findings point to hemolysis as a cause for anemia?elevated LDH, elevated bilirubin, jaundice, low haptoglobin
• Symptoms of plumber-vinson syndromeesophageal web and dysphasia, Iron deficiency anemia, glossitis
• Elevations in hemoglobin F, microcytic anemiabeta thalassemia
• X-ray findings with thalassemiacrew cut appearance
• Treatment for thalassemiatransfusions and iron chelation therapy as needed
• What is dactylitis?hand foot syndrome seen in children w sickle cell. Sausage digit inflammation
• This mimics pneumonia in sickle cell patientsacute chest syndrome
• These medications cause lupus like syndromesprocainamide, hydralazine, isoniazid
• Classic example drug that causes autoimmune hemolytic anemiamethyldopa. Also consider penicillin, cephalosporins, sulfa drugs and quinidine
• This test is positive in most autoimmune anemiasCoombs test
• Treatment for lead poisoning in children? Treatment in adults?succimer and children dimercaprol in adults. In severe cases use EDTA and dimercaprol
• Lab findings with sideroblastic anemia?elevated or normal Iron, ferritin, and total iron binding capacity
• This lab finding is elevated in anemia of chronic diseaseferritin
• This lab finding is the hallmark of spherocytosisincreased MCHC. Treat with splenectomy
• These drugs can cause aplastic anemiachloramphenicol, carbamazepine, sulfa drugs, zidovudine, Gold
• How do you treat a transfusion reaction if oliguria is presentIV fluids and diuretics
• This disorder prolongs PT, PTT, and bleeding timeDIC
• Treatment for DIC?treat the underlying cause
• Clotting test results with von Willebrand disease?high PTT and bleeding time
• Clotting test results with liver failure?high PT and PTT
• Upper respiratory infection then prolonged bleeding time?ITP
• TTP versus HUS?TTP has neuro stuff, HUS is kidney
• Treatment for TTP?plasmapheresis
• Heparin induced thrombocytopenia type I vs Type II?type I is non immune mediated and is not clinically significant. Type II increases risk of thrombosis and can cause a stroke for example
• Hemoglobin electrophoresis with B thalassemia minor would show increased ___increased hemoglobin A2
• Chronic kidney disease, EPO deficiency causing anemia. Give EPO then patient has microcytic anemia, why?give iron - EPO induced RBC production surge can precipitate iron-deficiency anemia
• Washed cells transfusion indicated forIgA deficiency, complement dependent autoimmune hemolytic anemia
• Leukoreduction could prefent what transfusion reaction?febrile nonhemolytic transfusion reaction, occurs 1-6 hours post transfusion
• Irradiated transfusion specimens indicated for which patients?bone marrow transplant recipients or acquired/congenital immunodeficient patients
• EPO injections can cause this side effectworsening of hypertension, we don’t know why really but just think of it as "thickening the blood"
• Low platelets but risk for arterial and venous thrombosis withheparin induced thrombocytopenia
• What is isosthenuria? Associated with what?inability to concentrate or dilute the urine, associated with sickle cell trait and disease
• Treatment for hair cell leukemiacladribine
• CHOP regimen treatment fornon hidgkin lymphoma
• 9/22 translocationCML, BCR ABL gene, defect with a non-receptor tyrosine kinase that forms.
• CF inheritence?autosomal recessive
• Treatment of TTP?emergent plasmapharesis (plasma exchange)
• If HIT is suspected, what do you do?stop and start factor 10 inhibitor (argatroban or fondaparinux for example)
• Distinguish between Iron deficiency anemia and thalassemia with this lab valueiron deficiency has an elevated RDW, normal in thalassemia
• Diagnosis of hereditary spherocytosis established byosmotic fragility test
• CD55 and CD59 testing forparoxysmal nocturnal hemoglobinuria
• Fever, flank pain, renal failure, hemolysis, and DIC within an hour of transfusion. What is this? How is diagnosis made?this is acute hemolytic transfusion reaction. Life treatening! Positive diret coomb's test, pink plasma ( hgb > 25) and cross type matching showing a mismatch.
• Angioedema, hypotension, difficulty breathing after transfusionIgA deficiency, anaphylactic shock to blood products
• Causes of warm agglutinin autoimmune hemolytic anemia?CLL, viral causes eg hiv, lupus, and drugs, specifically PENICILLIN.
• Causes of cold agglutinin autoimmune hemolytic anemia?mycoplasma or lymphoproliferative diseases (eg CLL)
• B12 and folate deficiency will cause increased levels of ____. B12 causes increased levels of ___homocysteine. Methylmalonic acid
• Any male or postmenopausal woman that comes in with iron deficiency anemia should be tested for this prior to starting iron supplementationoccult blood in stool
• Painless blisters, hyperpigmentation, possibly more hair. Associated with Hep C and can be triggered by alcohol or OCPs. What is this?porphyria cutanea tarda. Deficiency of uroporphyrinogen decarboxylase, enzyme in heme synthesis pathway. Discontinue triggering substance
• Hereditary telengiectasias, recurrent nosebleeds, and widespread AVMs. This can happen in the lungs and cause hemoptysis and right to left shunt shit so they get clubbing and stuffOsler Weber Rendu syndrome
• Kid with congenital marrow failure (pancytopenia), morphologic/skeletal abnormalities, hypopigmented skin spots, possible hearing issues, and usually macrocytic anemia. This is an autosomal recessive disorderFanconi anemia
• Isolated red cell aplasia associated with this type of tumorthymic tumor.
• Decreased leukocyte alkaline phosphatase scoreCML
• Normal RDW found in which anemias?the minor thalasemias
• Marked lymphocytosis vs marked neutrophilia, which cancer is which?CLL, CML
• Leukemoid reaction? How does this compare to CML?severe infection causing high leukocyte counts (>50,000). HIGH alkphos score in this, where it would be low in CML.
• Vitamin B6 deficiency causes what anemia that resembles iron deficiency but has increased iron levels and decreased TIBC?sideroblastic anemia. Defective heme synthesis. Happens with alcoholics and certain drugs (eg isoniazid)
• Lead toxicity workup after capillary (fingerstick) blood is drawn for initial screening?confirm with venous blood draw b/c the screening test has high false positives.
• Exclude these two infections before diagnosing ITPhepatitis C and HIV

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