Hematology is a subject that comes up daily in clinical practice, and is a favorite on the ABIM Internal Medicine board exam. Evaluation of anemia, as well as some other hematologic disorders, is paramount for an internist to master. I will try and provide five evidence-based pearls in this post that will help physicians understand some important concepts and avoid common pitfalls in the recognition and treatment of such hematological disorders.
Pearl #1: Patients on iron supplementation SHOULD NOT have positive guaiac tests.
Studies in vitro show ferric iron (Fe3+) will give a positive guaiac reaction and ferrous iron (Fe2+) does notIron is digested in the ferrous form and carried in the blood in the ferric formPatients on iron supplementation with positive guaiac require screening for identifying the source of gastrointestinal hemorrhageFerrous (Fe2+) iron does not cause positive guaiac tests in vivo
Pearl #2: The pentad of Thrombotic Thrombocytopenic Purpura (TTP) is not always present.
The pentad is: microangiopathic hemolytic anemia (MAHA), thrombocytopenia, renal abnormalities, neurologic abnormalities, and feverLess than 50% of patients have the complete pentadMeasurement of ADAMTS13 activity is not required to make the diagnosis; the diagnosis is clinicalThe gold standard treatment is plasma exchange and if not available you may use fresh frozen plasma as an alternative treatment
Pearl #3: Primary hemostasis disorders are a platelet dysfunction and secondary hemostasis disorders are a clotting factor disorder.
Primary hemostasis Disorders:
A result of platelet functionImmediate clottingPatients will have petechiae and purpuraAll will have elevated bleeding time (platelets don’t work) and normal PT/PTT (no problem with clotting factors)
Secondary hemostasis Disorders:
A result of clotting factorsDelayed clotting (help strengthen clots by fibrin formation)Patients will have hematomas and hemarthrosesAll will have normal bleeding time (platelets work fine) and abnormal PT (extrinsic pathway) and PTT (intrinsic pathway)
Pearl #4: Acute myelogenous leukemia (AML) type M3 has a good prognosis.
AML is the most common type of acute leukemia in adultsTypically M2 – M5 types are myeloperoxidase stain positive (Remember that PTU and micropolyangitis can also be positive)Auer rods are pathognomonic for AMLType M3 (promyelocytic) leukemia has t(15,17)The treatment of choice is all-trans retinoic acid (ATRA)The single most important prognostic factor in AML is cytogentetics: t(15;17) has a 70% 5 year survival and 33% relapse rate
Pearl #5: Anemia is the most common hematologic abnormality, so know it cold.
Iron deficiency anemia is the world’s most common cause of anemia
Iron deficiency anemia:
Low iron, transferrin saturation, and ferritinElevated TIBCTransferrin Receptor Index = transferrin receptor/Log Ferritin is the most sensitive assay for iron deficiency anemia (>2.0 = Iron Deficiency Anemia; <1.0 = Anemia of Chronic Disease)Treatment is PO iron → if no improvement after 6 weeks consider IV ironThe earliest lab to check after starting iron replacement is the reticulocyte count (Begins to increase at about 5 to 7 days)Ascorbic acid (vitamin C) supplementation increases absorption of ironMost iron is absorbed in the duodenumCeliac sprue can cause iron deficiency anemia: Best test is tissue transglutaminase antibody or antiendomysial antibody; Tx is a gluten free diet; Can see dermatitis herpetiformis associated with this entity
As I have stated in my high yield pearls to pass the boards: “Most commons” are emphasized on the ABIM curriculum, so it is good to know these disease processes.