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Hematology

FA review Round 1 2020

QuestionAnswer
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.
Created by: rakomi
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