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Hematology
Rx Review Round 4
| Question | Answer |
|---|---|
| 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
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