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Hematology
FA review Round 1 2020
| Question | Answer |
|---|---|
| Condition characterized by the complete absence of HbA1 in a child under 1 year old. | B-thalassemia major |
| Which type of hemoglobin unit is absent in B-thalassemia major? | HbA1 |
| Not the absence but a decrease in HbA1 is seen with which hematologic condition? | B-thalassemia minor |
| HbA1 is composed by: | 2-alpha and 2- beta |
| What is the featured histological finding in Acute Promyelocytic Leukemia (APL)? | Cytoplasmic Auer rods |
| What condition is characterized by the presence of promyelocytes with many cytoplasmic Auer rods? | Acute Promyelocytic Leukemia (APL) |
| What condition is strongly associated with APL? | DIC |
| What are the hematologic laboratory findings of DIC in a patient with ALP? | Prolonged PT/INR and PTT, and a decrease in Platelet count (PC) |
| What is the treatment for APL? | All-trans-retinoic acid |
| How does all-trans-retinoic acid act in the treatment for APL? | Allow malignant promyelocytes bot overcome their maturation block and mature into myelocytes, metamyelocytes, and finally neutrophils |
| What is the most common inherited bleeding disorder? | von Willebrand disease |
| What is the mode of inheritance of vWD? | Autosomal dominant |
| A child with 1 affected parent with vWD, has how much chance to develop this condition as well? | 50% |
| What is the characteristic symptom of Malaria? | Relapsing fever |
| How is Malaria clinically presented? | Relapsing fever, headaches, anemia, and splenomegaly |
| What pathogen infection causes Malaria? | Plasmodium species |
| What disease should be suspected in a person that has traveled to Africa? | Malaria |
| PBS shows presence of parasites in Red Blood cells. Dx? | Malaria |
| What is the main virulence factor in Salmonella causing Osteomyelitis? | Capsule |
| Why are functional asplenia more likely to develop Salmonella osteomyelitis? | Asplenic patients are more susceptible to encapsulated bacteria |
| What hematologic condition is associated with functional asplenics? | Sickle cell patients |
| What is the definitive treatment for Hereditary Spherocytosis? | Splenectomy |
| Which hereditary hematologic condition is treated with a Splenectomy? | Spherocytosis |
| Description of RBC in Spherocytosis | Spherical with consequently impaired capacity for gas exchange and seen with anemia |
| What condition is featured with rigid spherical RBCs that have decreased capacity for gas exchange and lead to anemia? | Spherocytosis |
| What type of anemia is caused by Hereditary Spherocytosis? | Intrinsic hemolytic anemia |
| What are the main findings fo Spherocytosis? | 1. Splenomegaly 2. Aplastic crisis (Parvo B19 infection) |
| What pathogen is responsible for the aplastic crisis seen clinically in Hereditary Spherocytosis? | Parvo B19 |
| List of Intrinsic Hemolytic anemias: | 1. Hereditary Spherocytosis 2. G6PD deficiency 3. Pyruvate kinase deficiency 4. Paroxysmal Nocturnal Hemoglobinuria 5. Sickle cell anemia 6. HbC disease |
| Clinical findings of G6PD deficiency : | Back pain, hemoglobinuria after few days of oxidant stress (drugs, illness, infection, fava beans) |
| What condition is defined as the Hemolytic anemia in Newborn? | Pyruvate kinase deficiency |
| What type of anemia is seen with Pyruvate kinase deficiency? | Intrinsic hemolytic anemia |
| G6PD deficiency includes what type of anemia? | Intricisc hemolytic anemia |
| What is the typical triad seen in Paroxysmal Nocturnal Hemoglobinuria? | 1. Coombs (-) hemolytic anemia 2. Pancytopenia 3. Venos thrombosis (eg. Budd-Chiari syndrome) |
| Coombs (-) or (+) hemolytic anemia, in Paroxysmal Nocturnal Hemoglobinuria? | Coombs (-) hemolytic anemia |
| What is Pancytopenia? | Condition that occurs when a person has low counts for all three types of blood cells: red blood cells, white blood cells, and platelets. |
| Which cells are in low counts in Pancytopenia? | Red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia) |
| What key urine condition or clue is associated with Paroxysmal Nocturnal Hemoglobinuria (PNH)? | Pink/red urine in the morning |
| What conditions or illnesses are associated with Paroxysmal Nocturnal Hemoglobinuria? | 1. Aplastic anemia 2. Acute leukemias |
| Disease in which the body fails to produce blood cells in sufficient numbers | Aplastic anemia |
| What type of hypersensitivity is seen with ABO-incompatible blood? | Type 2 hypersensitivity |
| Type II hypersensitivity reaction, in which IgG antibodies cause complement lysis of RBCs. Dx? | ABO-incompatible blood |
| What are the common symptoms seen with a ABO-incompatible blood disease? | Hypotension, tachycardia, and end-organ damage |
| What pathogen causes Visceral Leishmaniasis? | Leishmania donovani |
| What is the bug that transmits Leishmania donovani? | Sandfly |
| What pathogen is transmitted by the Sandfly? | Leishmania donovani |
| Clinical features of Visceral Leishmaniasis? | 1. Kala azar ( "Black disease") 2. Hepatosplenomegaly 3. Malaise and decrease weight 4. Pancytopenia |
| What is Kala azar? | Black disease; represented by the gray discoloration of the hands, feet, abdomen and face |
| A person with a a grayish discoloration of hand, feet, face, and areas of abdomen, should raise suspicion of which condition? | Visceral Leishmaniasis which is seen with Kala azar or "Black disease" |
| What is another way to refer to Kala azar? | Black disease |
| Proliferation of circulating Myeloblasts in peripheral smear. Dx? | AML |
| What are common risk factor of AML? | Exposure to alkylating agents, Radiation, and Down syndrome |
| What is the MC presentation of AML? | DIC |
| What is Primary Myelofibrosis? | Chronic myeloproliferative disorder caused by an overproduction of myeloid cells in bone marrow often as result of JAK2 mutation |
| Which is the most common associated mutation of myelofibrosis? | JAK2 |
| A JAK2 mutation most likely indicates what type of disorders? | Myelofibrosis |
| What is an important condition developed due to Primary Myelofibrosis depletion of bone marrow? | Extramedullary Hematopoiesis |
| What organs are the main ones to carry Extramedullary Hematopoiesis? | Liver and Spleen |
| RBC seen in Myelofibrosis? | Teardrop or Dacrocyte |
| Why is Primary Myelofibrosis seen with a Dry Tap? | Due to depletion or absence of bone marrow |
| What virus is strongly associated with the pathogenesis of Burkitt lymphoma? | EBV |
| EBV is a (composition): | Linear, double-stranded DNA virus |
| What is the function or role of G6PD? | Catalyzes NADP to its reduced form NADPH |
| Which is the reduced form, NADP or NADPH? | NADPH |
| What is the relation between NADPH and G6PD deficiency? | In G6PD deficiency the NADPH levels are too LOW to maintain glutathione in a reduced state during oxidative stress |
| In G6PD, is there a buildup or accumulation (increased levels) of reduced glutathione or oxidized glutathione? | Oxidized glutathione |
| What damages the hemoglobin in G6PD deficiency? | Buildup (increased level) of oxidized glutathione |
| Histological feature of Vitamin B12 deficiency? | Increased nuclear lobulations in neutrophils, also known as Hypersegmented neutrophils |
| What is the common treatment of Vitamin B12 deficiency? | Injections of vitamin B12 |
| Lab findings of vWD: | 1. Prolonged bleeding time 2. 50% of patients have elevated PTT |
| Why do half of persons with vWD present with elevated BT, and also PTT? | Prolonged PTT is due to the fact that vWF is the carrier protein of factor 8 |
| vWF is the carrier protein for which factor? | Factor 8 |
| Bleeding time is a: | Measure of platelet function |
| All platelet disorders will have prolonged ___________________. | Bleeding time (BT) |
| Hemophilias have normal, reduced, or prolonged bleeding times? | Normal |
| How is Sickle cell anemia associated with development of Macrocytic anemia? | Increased erythropoiesis and elevated demand of Folate, can predispose to a Folate deficiency, which in turn develops a macrocytic anemia |
| How is CML commonly manifested? | 1. Left-shifted neutrophilia and, 2. Low LAP activity |
| What hematologic condition is characterized with neutrophilia and low LAP activity? | CML |
| LAP activity in CML is low or high? | Low |
| Associated protein of CML? | BCR-ALB fusion protein |
| The bcr-alb fusion protein is a product of an: | Oncogene |
| Condition typically treated with Imatinib? | CML |
| How does Imatinib treats CML? | Targeted to a small molecule inhibitor that blocks the function of bcr-alb. |
| What protein's function is blocked by the use of Imatinib? | BCR-ALB fusion protein |
| Example of an oncogene byproduct: | BCR-ALB fusion protein |
| What are the functions of vWF? | 1. Platelet adhesion 2. Protein carrier for factor VIII (8) |
| (+) or (-) Direct Coombs test in Autoimmune Hemolytic anemias? | (+) Direct Coombs test |
| What does it mean to yield a (+) Direct Coombs test? | Antibodies present to the patient's RBCs |
| What are common associations or values/measures of Autoimmune hemolytic anemia? | 1. (+) Direct Coombs test 2. Elevated Lactate dehydrogenase level 3. Elevated Indirect bilirubin level 4. Low levels of Haptoglobin |
| Are the Haptoglobin levels in Autoimmune hemolytic anemia, elevated or decreased? | Decreased |
| What is possible diagnosis of a patient with indirect hyperbilirubinemia, low haptoglobin ,and (+) direct Coombs test? | Autoimmune Hemolytic anemia |
| What is haptoglobin? | Protein that binds to free hemoglobin, released from erythrocytes with high affinity, and thereby inhibits its deleterious oxidative activity. |
| What RBC secreted protein is associated to inhibit the oxidative activity to free hemoglobin? | Haptoglobin |
| Which genetic condition is strongly associated to Acute Leukemias? | Down syndrome |
| What are common acute leukemias? | ALL and AML |
| How do acute leukemias develop symptoms? | Bone marrow infiltration by tumor cells, leading to depression of other cell lines |
| What is the pathogenesis of symptoms seen in acute leukemias? | Fever, loss of weight, and anemia, resulting in fatigue and thrombocytopenia that causes easy bruising |
| Failure to recover blood pressure despite aggressive hydration. Dx? | Septic shock |
| Hemodynamics seen in Septic shock: | 1. Decreased PCWP 2. Decreased PVR 3. Increased CO |
| PCWP and PVR are both, decreased or increased, in Septic Shock? | Decreased |
| Which hemodynamic measure is increased in Septic shock? | Cardiac output (CO) |
| What is Paroxysmal Nocturnal Hemoglobinuria? | Rare acquired disorder caused by PIG-A anchor mutation, which leads to intravascular hemolysis and hemosiderin in the urine |
| What is the associated mutation of Paroxysmal Nocturnal hemoglobinuria? | PIG-A anchor mutation |
| What compound is found in the urine of a patient with Paroxysmal Nocturnal Hemoglobinuria? | Hemosiderin |
| What causes the hemosiderin in urine in patient with Paroxysmal Nocturnal Hemoglobinuria? | Red/pink color of urine in the morning |
| What medication is used to treat Paroxysmal Nocturnal Hemoglobinuria? | Eculizumab |
| A patient on Eculizumab, is likely to be diagnosed for: | Paroxysmal Nocturnal Hemoglobinuria |
| What is the MOA of Eculizumab? | Complement protein C5 |
| What is Hemophagocytic Lymphohistiocytosis (HLH)? | Affects infants, and is caused by hyperinflammation and proliferation of macrophages that improperly phagocytize host blood cells |
| Clinical features and symptoms of Hemophagocytic Lymphohistiocytosis? | Anemia, Thrombocytopenia, hypertriglyceridemia, increased ferritin, hepatosplenomegaly, jaundice, fever, and lymphadenopathy. |
| What is a distinguishing serum level of HLH? | Hypertriglyceridemia |
| What is an Autologous graft? | Transplantation of the patient's own tissue from one site to another |
| Which graft transplantation is least likely to be rejected? | Autologous graft |
| What type of graft transplantation is the most common used to bone marrow transplantation in a patient with Hodgkin lymphoma? | Autologous graft |
| What are the two main causes of anemia in a patient with Lupus? | 1. Anemia of chronic disease 2. Autoimmune Hemolytic anemia |
| What are some important histological and lab features of Autoimmune hemolytic anemia? | 1. Type II Hypersensitivity reaction 2. Increased reticulocyte count, spherocytes on PBS, and (+) Direct Coombs test |
| What type of hypersensitivity reaction is Autoimmune Hemolytic anemia? | Type II hypersensitivity reaction |
| What type of RBCs are seen or associated with Autoimmune hemolytic anemia? | Spherocytes |
| Which known hemolytic diseases are seen with PBS (+) for spherocytes? | Hereditary Spherocytosis and Autoimmune Hemolytic anemia |
| How is LMWH administered (route)? | Subcutaneously |
| MOA of LMWH | Predominantly potentiates antithrombin III, which inhibits Factor Xa |
| True or False. LMWH does not require PTT monitoring. | True |
| What protein or compound is potentiated by LMWH? | Antithrombin III |
| What factor is inhibited by Antithrombin III? | Factor Xa |
| Which protein or compound is known to inhibit the actions of Factor Xa in coagulation cascade? | Antithrombin III |
| Serious sequelae of gram (-) sepsis and describes a microangiopathy and consumption coagulopathy. Dx? | DIC |
| What type of angiopathy is associated with DIC, micro- or macroangiopathy? | Microangiopathy |
| What is CLL? | Low-grade lymphoproliferative disorder in older patients |
| Which population is most likely to develop CLL? | Elders |
| How is CLL commonly clinically presented? | Lymphadenopathy, splenomegaly, infections, and autoimmune hemolytic anemia |
| What anemia is seen in patients with CLL? | Autoimmune Hemolytic anemia |
| What is the most common adult leukemia? | CLL |
| CLL is positive for which CDs? | CD5+, CD23+, and CD20+ |
| CD23+ leukemia | CLL |
| What two organ transplant lead most commonly to GVHD? | Bone marrow and liver |
| What is the pathogenesis of GVHD? | Grafter donor immunocompetent T cells that rejet Host cells |
| What type of graft rejection is due to Donor T-cells rejecting or attacking Recipient host cells? | GVHD |
| What organs are mostly targeted by Donor T cells in GVHD? | Gut, skin, and liver, which cause diarrhea, maculopapular rashes, and juancide, as well as hepatosplenomegaly |
| What is accumulated (substrate) in Lead poisoning? | Aminolevulinic acid (ALA) |
| What condition is characterized by accumulation of ALA? | Lead poisoning |
| What enzyme is inhibited by lead poisoning? | ALA dehydratase |
| If ALA dehydratase is inhibited: | It accumulates ALA, and is most likely due to Lead poisoning |
| What are the two factors that drive Angiogenesis? | VEGF and FGF |
| What process is driven by VEGF and FGF? | Angiogenesis |
| What is the function of VEGF? | Increases endothelial cell proliferation and leads to new capillary formation |
| What is the role of FGF? | Involved in endothelial cell proliferation like VEGF, but unlike VEGF, it involved in addition processes such as hematopoiesis and wound healing |
| What additional roles are carried by FGF, that are not seen in with VEGF? | 1. Hematopoiesis 2. Wound healing |
| Which growth factor, FGF or VEGF, is involved in hematopoiesis, endothelial cells proliferation, and wound healing? | FGF |
| What function or functional property is seen with FGF and VEGF? | Endothelial cell proliferation leading to new capillary formation |
| List of inactivated vaccines: | 1. Hepatitis A 2. Typhoid 3. Rabies 4. Intramuscular Influeza 5. Polio (Salk) |
| Which polio type is an inactivated vaccine? | Salk polio |
| IM influenza is a live or inactivated vaccine? | Inactivated vaccine |
| Hepatitis A, Rabies, and IM influenza, as well as Salk Polio are examples of: | Inactivated vaccine |
| What immunity is elicited by Inactivated vaccines? | Humoral immunity only |
| If the vaccine elicits a humoral immunity response only, it is safe to say it is a ________________ vaccine. | Inactivated vaccine |
| Recombinant anti-CD20 antibody that is now used in Large B-lymphocyte lymphoma. | Rituximab |
| What are some associated adverse effects of Rituximab? | Low blood pressure, bronchospasms, chills, and decreased blood platelets, lymphocytes, and neutrophils |
| What type of neoplasm is commonly treated with Rituximab? | Large B-lymphocyte lymphoma |
| What are common causes of Iron deficiency anemia in the elderly? | 1. Nutritional deficiency of iron 2. Chronic bleeding |
| What is a common malignancy in elderly that leads to Iron deficiency anemia? | Colorectal malignancy |
| A 73 year old man with Colorectal cancer, is probably also going to develop what type of anemia? | Iron deficiency anemia |
| Other than the immediate removal of heparin admisnistartion, what other treatment option is done in HIT? | Direct Thrombin inhibitor administration |
| What are some examples of Direct Thrombin inhibitors? | Argatroban, Bivalirudin, and Degatraban |
| What condition is treated with administration of a Direct Thrombin inhibitor? | HIT |
| Which MHC is downregulated by cancer cells? | MHC I |
| How is MHC I in cancer? | Downregulated by cancer cells in an attempt to evade destruction by CD8+ T cells |
| After downregulation of MHC I class moleculares by cancer cells, what is the primary cell to combat cancer? | Natural killer cells |
| When do NK cells become the primary defense against cancer cells? | After the downregulation of MHC I class molecules. |
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rakomi
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