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Hematology

Rx Review Round 4

QuestionAnswer
What is a common measure of FDP in Thrombophilia? Increased D-dimer
What is the MCC of Inherited Thrombophilia? Decreased cleavage of factor Va by Protein C
What are some common Thrombotic states? DVT, PE, and DIC
PPV = TP / (TP + FP)
In Hardy-Weinberg population, what does each stand for: p= q= 2pq= = Frequency of A (dominant allele) = Frequency of a (recessive allele) - Frequency of HETEROZYGOSITY (Aa), or carrier frequency
Iron deficiency anemia is the MCC of? Microcytic, hypochromic anemia
What are the MCCs of Iron deficiency anemia? 1. Nutritional deficiency of Iron 2. Chronic bleeding
What is Staphylococcal Toxic Shock Syndrome? Massive cytokine release in response to EXOTOXIN
What is the clinical manifestation of Staphylococcal Toxic Shock syndrome? Fever, Hypotension, and involvement of multiple organ systems
What is the skin hallmark of Staphylococcal Toxic Shock Syndrome? Palmar and Plantar Desquamation 2 weeks after onset of disease
Down Syndrome patients are at higher risk of developing which type of Leukemia? ALL and AML
What is another term used for "Black Disease"? Kala azar
What kind of organism transmit Leishmania donovani? Sandfly
What disease is caused by Sandfly transmission? Leishmaniasis caused by Leishmania donovani
What is the clinical presentation of Kala azar? Hepatosplenomegaly, malaise, Pancytopenia, and Weight loss.
Which a commonly used immunosuppressant used to prevent GVHD in Bone marrow transplant patients? Cyclosporine
What is a serious side effect of Cyclosporine? Nephrotoxicity
What drug is used to prevent nephrotoxicity caused by use of Cyclosporine? Mesna
What kind of test are to be tested prior to starting a patient on Cyclosporine? Renal function testing
What are two common nutritional deficiencies that cause Megaloblastic anemia? Vitamin B12 and Folate deficiency
What is an important function of Vitamin B9? Works as intermediate in the transfer of 1-carbon units
Which water soluble vitamin is involved in the transfer of 1 carbon units? Vitamin B-9
Which bleeding disorder is associated with thrombocytopenia and not with systemic disease? Immune thrombocytic Purpura (ITP)
What are the clinical features seen in ITP? Easy bruising, bleeding through skin or mucous membranes, and LOW PLATELET count
What does the PBS of ITP show? Increased megakaryocytes
What type of inheritance is VW disease? Autosomal dominant
What is deficient in vW disease? Von Willebrand Factor
What is the common function of vWF? 1. involvelved in platelet adhesion 2. Acts as carrier molecule for factor VIII
What is seen in labs of vW Disease? 1. Reduced Ristocetin-induced platelet aggregation assay 2. Prolonged PTT (intrinsic)
What is the genomic description of EBV? Linear, double-stranded DNA virus with an Enveloped
What is the MCC organism of Infectious mononucleosis? EBV
What are the clinical features of Infectious mononucleosis? Flu-like symptoms, Cervical lymphadenopathy, and Splenomegaly.
Which immunoglobulins are involved in initiation of the Classical Complement pathway? IgG and IgM
In classical complement pathway, where is the attachment site in the IgG or IgM? CH2-component of the Fc Fragment, which is above the macrophage binding site
Which specific molecule can be used as an opsonin in order to improve macrophage function in the Classical Complement pathway? C3b
Where is macrophage binding in the immunoglobulin? CH3-component of the Fc fragment
What is the hallmark of cancer that has mutations in the genes that regulate apoptosis? Evade Apoptosis and continue to replicate
What is a common cancer associated with Evasion of Apoptosis? Follicular B cell lymphoma
What are some common genes that regulate apoptosis? TP53 and BCL-2
What is the MCC of osteomyelitis in a Sickle cell patient? Salmonella infection
What is the main virulence factor for developing osteomyelitis? The CAPSULE shared by Staph aureus and Salmonella
What translocation is strongly associated with AML? t(15;17)
What is the most common age for a patient to develop AML? 31 years old
Which bleeding disorder is associated with AML? DIC
What are the clinical features of DIC? Spontaneous bleeding, purpura, elevated PT and PTT, and decreased fibrinogen
How are the levels of fibrinogen in DIC? Decreased
What is a key component in the Blood smear inspection of AML? Increased myeloblast with Auer rods
What are the two most common type of transplantation that lead to GVHD? Bone marrow and Liver
Why are Bone Marrow and Liver the most common type of transplanted organs to develop GVHD? They are rich in lymphocytes
What is the pathogenesis of GVHD? Grafted Donor Immunocompetent T-cells that reject HOST cells
What are the main organs affected in GVHD? Gut, skin, and the liver
What are the most common clinical features of GVHD? Diarrhea, maculopapular rash (dermatitis), jaundice, and hepatosplenomegaly.
What are the tree types of Host vs Graft disease? Hyperacute, acute, and chronic rejection
What is the pathogenesis of Hyperacute Host vs Graft rejection? Pre-existing antibodies react to donor antigen
What is the difference in the pathogenesis (excluding time of onset) between Humoral Acute transplant rejection and Hyperacute transplant rejection? In Humoral Acute Transplant rejection, the antibodies are produced AFTER the transplant, while in Hyperacture, the antibodies are PRE-EXISTING.
What kind of rejection is described by CD4+ and CD8+ T-cells been activated against donor MHCs? Cellular Acute Transplant rejection
What is the pathogenesis of Chronic Transplant rejection? CD4+ T-cells respond to recipient APCs presenting donor peptides including allogeneic MHC
Which type of Host vs Graft rejection is(are) Type 2 Hypersensitivity? Hyperacute and Chronic
Which two Host vs Graft rejections are considered type 4 Hypersensitive reactions? Cellular acute and Chronic rejections
Vasculitis of graft vessels with dense interstitial lymphocytic cellular infiltrate, is seen in ? Cellular Acute Transplant rejection
Which type of rejection is dominated by arteriosclerosis? Chronic Transplant rejection
Chronic transplant rejection is manifested clinically by: Recipient (host) T-cells react and secrete cytokines --> : - vascular smooth muscle proliferation - Interstitial fibrosis
What kind of cells is mostly associated with Non-Hodgkin lymphomas? B cells
What are the three main T-cell derived Non-Hodgkin lymphomas? T-cell lymphoblastic leukemia/lymphoma, Mycosis fungoides, and Sezary syndrome
What are the classic symptoms seen in both Hodgkin's and Non-Hodgkin lymphomas? Decreased weight, anemia, generalized lymphadenopathy, and hepatosplenomegaly
What is a histological characteristic of Hodgkin lymphomas? Reed-Sternberg cells
What is a common feature of Non-Hodgkin lymphomas, that distinguish them from Hodkin? Involves multiple lymph nodes, and extranodal involvement is commonly seen.
What are the associated autoimmune diseases and viruses with Non-Hodgkin lymphomas? HIV, EBV, and HTLV
What is the most common form of Non-Hodgkin lymphoma in adults? DLBCL
What are the most important risk factors for the development of DLBCL? 1. Increasing age 2. Immunosuppressants
What are the genetics involved in DLBCL? Alteration sin BCL-2 and BCL-6
What is the function or responsibility of G6PD? The recycling of NADP+ into NADPH
What is the result of deficiency G6PD enzyme? Inability to recycle GLUTATHIONE, ultimately leading to OXIDATIVE stress ----> Hemolytic anemia
What are clinical features of G6PD deficiency? Fatigue, Dark urine, and jaundice
What is the mode of inheritance of G6PD deficiency? X-linked recessive
What is a common and serious complication of Allogeneic blood or Bone Marrow transplant? Acute GVHD
What is the pathogenesis of Hemophagocytic Lymphohistiocytosis? Affects infants and is caused by hyperinflammation and proliferation of MACROPHAGES that improperly phagocytize host blood cells
Which condition is described by : macrophages eating infant's good red blood cells? Hemophagocytic Lymphohistiocytosis
What is the common presentation of Hemophagocytic Lymphohistiocytosis? Anemia, thrombocytopenia, hypertriglyceridemia, hepatosplenomegaly, jaundice, fever and lymphadenopathy
Extremely elevated ferritin levels in an infant can be indicative of? Hemophagocytic Lymphohistiocytosis
HIT is characterized by: - Extreme Thrombocytopenia and increased risk of thrombosis
What is the common treatment for prevention of thrombosis in HIT? Direct Thrombin inhibitors
What are some common Direct Thrombin inhibitors? Argatroban, Bivalirudin, and Dabigatran
Which is the only oral direct thrombin inhibitor? Dabigatran
What is the use for Protamine sulfate? ONLY used to REVERSE the effects of Heparin in cases of overdose associated with bleeding.
What is Uremic Platelet dysfunction? Condition seen in Renal insufficiency patients, that have unfiltered uremic toxins affecting platelet function.
What are the hematologic times seen in Uremic Platelet dysfunction? - Increased Bleeding Time, but normal PC count and Normal PT/ aPTT
What are the two most significant CBC findings in Thalassemia? 1. Increased RBC 2. Normal RDW
What kind of RBC cell is seen in Thalassemias? Target cells
What is the description of a Target cells? Red cell with a condensation of hemoglobin in the center
A high reticulocyte count is seen in which type of anemias? Those due to cell destruction such as Spherocytosis
A low Reticulocyte count will indicate anemias caused by? A production problem
A deficiency in Vitamin K will produced prolonged ______ and _______. PT and PTT
How are BT and PC levels affected in Vitamin K deficiency? Unaffected (normal levels)
What mnemonic is used to remember that PTT is associated with the Intrinsic Coagulation Pathway? Play Table Tennis Inside
Affected extrinsic pathway will result in a prolonged ___________. PT
Which is the coagulation factor associated with a prolonged PT? Factor 7, as it is the only one in the Extrinsic Coagulation pathway.
Why is there a hypochromic RBC in Iron deficiency anemia? Due to decreased Heme synthesis
By what age is an infant mostly depleted of all iron storages? By 6 months of age
Why is it important to provide IRON supplemented milk to a baby that is 6 months of age? All iron storage is gone, and baby otherwise may suffer of Iron deficient anemia
What are the Increased lab levels in Iron deficient anemia? TIBC, free erythrocyte protoporphyrin, and RDW
What are common symptoms of Iron Deficient anemia? 1. Fatigue, and conjunctival pallor 2. Pica (craving and compulsive ingestion of non-food substances) 3. Spoon nails (Koilonychia)
Thymomas are associated to which Paraneoplastic syndromes? 1. Myasthenia gravis 2. Pure RBC aplasia 3. Hypogammaglobulinemia
What is Pure RBC aplasia? Autoimmune disorder causing proliferation of RBC precursors in the bone marrow
What type of genetic phenomenon is associated with B-Thalassemia? Allelic Heteroplasmy
What is Allelic Heteroplasmy? Phenomenon in which different mutation at the same locus produce similar phenotype.
Lymphoblastic lymphomas are: Non-Hodgkin lymphomas, dx by increased lymphoblastic proliferation in the bone marrow
What kind of population is most likely to develop LymphoBLASTIC lymphomas? Children and young adults
What is the hormone that stimulates RBC production? EPO
What is the main function of EPO? Stimulation of RBC production
What kind of anemia is seen in those with renal failure? Normocytic anemia with normal platelet and WBC counts
What is the main reason of anemia in renal patients? EPO deficiency
What are the broad features of Lead poisoning? Fatigue, abdominal pain, Peripheral neuropathy, and headache
How does lead poisoning causes anemia? It produces microcytic anemia by interrupting HEME synthesis
HEME synthesis disruption anemia is seen with ______________. Lead poisoning
What is the most classic histological finding of RBCs in lead poisoned patients? Basophilic stippling
What are two complications of Inherited Heme synthesis problems? 1. Acute Intermittent Porphyria 2. Porphyria Cutanea Tarda (photosensitive)
HIT is? Immunologic reaction to Heparin
HIT Type 1: - Not an antibody-mediated response - Platelets do not fall below 100,000/mm3
HIT Type 2: - Due to formation of new Heparin antibodies - Platelets fall below 100,000/mm3
What are the most serious risks in HIT? Bleeding and Intravascular thrombosis
What is a common RENAL complication of DIC? Diffuse Cortical Necrosis
What is the common presentation of Diffuse cortical necrosis? Anuria, Gross hematuria, and flank pain.
What is the most common treatment option for a patient it complicated Diffuse Cortical Necrosis (DCN) from DIC? Dialysis
What is the antimetabolite of 6-MP? Azathioprine
What is the mode of action of Azathioprine? Interferes with Purine Nucleotide Synthesis
Diagnosis of a patient with deficiency in clotting factor 8? Hemophilia A
What is the most immediate treatment for Hemophilia A? Infusion of Factor 8
What is the clinical presentation of Hemophilia A patients? Easy bruising, hemarthrosis, and bleeding caused by oral injury
Anti-Rh antibodies are: Found in Positive INdirect Coombs test in Rh negative women who have CARRIED a Rh + fetus previously.
What are the PENTAD of symptoms associated with TTP? 1. MAHA 2. Thrombocytopenia 3. Neurologic abnormalities 4. Renal abnormalities 5. Fever
RBCs in blood smear of a TTP patient will most likely show? Broken, damaged RBCs
TTP is due to a deficiency in which enzyme? Metalloprotease (ADAMTS13)
The deficiency of ADAMTS13 will most likely develop in? TTP
Reason of large multimers of vWF in TTP? Due to deficiency of ADAMTS13, a metalloprotease, which cleaves vWF multimers.
Which form of hemoglobin is associated with lower Oxygen affinity? Taut form
The Taut form of Hemoglobin is predominant in _______________ tissue. Peripheral
Why is Taut form of Hb more readily to release Oxygen? Lower O2 affinity leads to easier release.
Which tissue is often associated with R-form of Hemoglobin? Pulmonary capillaries
Which form of hemoglobin has higher oxygen affinity? Relaxed (R) form
An area or tissue that is highly required to "pick up" oxygen is going to have which form of Hemoglobin? Relaxed (R) form
How many affected copies of the affected allele are needed to express disease in an AD disorder? 1
1 -A = the frequency (Hardy-Weinberg) of a, given the value A.
Equation used to find the frequency of affected allele in an AD disorder? A +Aa = p(2) + 2pq
What are the two main Growth Factors involved in ANGIOGENESIS? 1. VEGF (Vascular Endothelial Growth Factor) 2. FGF (Fibroblast Growth Factor)
In which function are VEGF and FGF both involved? Endothelial cell proliferation
What are specific functions or involvements of FGF, which VEGF is not? 1. Hematopoiesis 2. Wound healing
Which is an aggressive non-Hodgkin lymphoma associated with EBV infection? Burkitt Lymphoma
What is seen in biopsy of an Burkitt Lymphoma? Highly mitotic, basophilic lymphocytes surrounding clear zones of macrophages .
What are characteristics clinical symptoms of Burkitt lymphoma? Anorexia, Constipation, and Abdominal pain
How is LMWH given most commonly? Subcutaneously
What is the DIRECT action of LMWH? Potentiates antithrombin III
What is the INDIRECT action of LMWH? Inhibition of Factor Xa
Why is it safe to use LMWH anticoagulant therapy on OUTPATIENTS? It does not require PTT monitoring
Created by: rakomi