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FA Hemato Patho
Question | Answer |
---|---|
Where is Erythropoietin released? | Endothelial cells of peritubular capillaries |
What cell is detected with methylene blue and has thread like RNA filaments? | Reticulocytes (immature erythrocyte) |
In anemia, the reticulocyte count is a marker of what? | Effective erythropoiesis (bone marrow response to anemia) |
What is the formula for corrected reticulocyte count? | Hct / 45 x reticulocyte count |
What is the corrected reticulocyte count in hemolytic anemia? | More than 3% |
Give 4 examples of diseases with corrected reticulocyte count below 3%. | Acute blood loss, Aplastic anemia, Anemia of Chronic disease, Chronic renal failure (decresed EPO). |
What does MCV measure? | MCV = Mean Corpuscular Volume Measures the average volume of RBC |
What does MCHC measure? | MCHC = Mean Corpuscular Hemoglobin Concentration Measures the average Hb concentration in RBCs. |
Give examples of diseases with decresed MCHC. | All microcytic anemias : Iron deficiency anemia, Anemia of Chronic disease, Thalassemia, Lead poisoning. |
What does RDW measure? | RDW = RBC Distribution Widith Measures the variation in size of RBC |
Which disease have decresed serum ferritin? | Iron deficiency anemia |
Which diseases have increased serum ferritin? | Anemia of Chronic disease, Iron overload disease. |
Which diseases have increased serum iron? | Iron overload disease |
Which diseases have decreased serum iron? | Iron deficiency anemia, Anemia of Chronic disease |
How does TIBC level relate to concentration of transferrin? | Increased transferrin = increased TIBC |
How does TIBC level relate to serum ferritin level? | Decreased ferritin stores cause increased synthesis of transferrin (which is proportional to TIBC level). Vice versa. |
What does Iron saturation represent? | Represents percentage of binding sites on transferrin occupied by iron. |
RBC : Spherocytes are seen in which disease? | Hereditary spherocytosis, Autoimmune hemolytic anemia (Warm/Cold) |
RBC : Eliptocytes are seen in which disease? | Hereditary eliptocytosis |
RBC : Helmet cells / Schistocyte is seen in which disease? | DIC, TTP/HUS, traumatic hemolysis |
RBC : Bite cells are seen in which disease? | G6PD deficiency |
RBC : Teardrop cell is seen in which disease? | Myeloid metaplasia with myelofibrosis |
RBC : Acanthocyte (spur cell) is seen in which disease? | Liver disease, abetalipoproteinemia |
RBC : Target cell is seen in which disease? | HALT HbC disease, Asplenia, Liver disease, Thalassemia |
RBC : Burr cell is seen in which disease? | TTP/HUS |
RBC : Basophilic stippling is seen in which disease? | TAIL Thalassemia, Anemia of Chronic disease, Iron deficiency, Lead poisoning |
RBC : Heinz bodies are seen in which disease? | Alpha thalassemia, G6PD deficiency Mech : Oxidation of iron from ferrous to ferric -> Hb ppt -> RBC membrane dmg -> bite cells |
RBC : Howell-Jolly bodies | Functional hyposplenia, Asplenia (Basophilic nuclear remnants in RBC) |
List 5 causes of Microcytic anemia. | Iron deficiency, Thalassemia, Lead poisoning, Sideroblastic anemia. Anemia of Chronic disease |
Dx : Hypersegmented neutrophils. | Megaloblastic anemia, caused by vit B12 and/or folate deficiency. |
What does decreased haptoglobin and increased LDH indicate? | RBC hemolysis |
What is the test used to distinguish between immune vs non-immune mediated RBC hemolysis? | Direct Coombs' test |
What test can you perform to determine the cause of B12 deficiency? | Schilling test |
Dx : Macrocytic anemia with methylmalonic aciduria. | Vit B12 deficiency |
Dx : Macrocytic anemia with achloryhydria. | Vit B12 deficiency |
Where is Vit B12 reabsorbed in the GI tract? Where is Folate reabsorbed in the GI tract? Where is Iron reabsorbed in the GI tract? | Vit B12 = terminal ileum Folate = jejunum Iron = Duodenum |
Dx : Chronic alcoholism, increased serum iron, anemia. | Sideroblastic anemia |
Dx : Isoniazid therapy is associated with what vitamin deficiency? leading to what anemia? | B6 deficiency leading to sideroblastic anemia |
Dx : Ringed sideroblast. Microcytic anemia. | Sideroblastic anemia |
Dx : Coarse basophilic stippling. Microcytic anemia. | Lead poisoning |
Dx : Causes includes paint, batteries, and a type of poisoning. Microcytic anemia. | Lead poisoning |
Dx : Abdominal pain with diarrhea, growth retardation and encephalopathy in children | Lead poisoning |
Name 2 enzymes which lead poisoning denatures. | Ferrochelatase, and ALA dehydratase |
Name 2 accumulated substrate in urine in Lead poisoning. | Coproporphyrin, ALA |
Name the enzymes affected in Acute intermittent porphyria. | Uroporphyrinogen I synthase |
Name the accumulated substrate in urine in Acute Intermittent porhyria. | Porphobilinogen, ALA |
Name the enzyme affected in Porphyria cutanea tarda. | Uroporphyrinogen decarboxylase |
Name the accumulated substrate in urine in Porphyria cutanea tarda. | Uroporphyrin (tea-coloured) |
What are the symptoms of Acute intermitten porphyria? | 5P Pain in abdomen, Pink urine/Portwine urine Polyneuropathy, Psychological disturbances, Percipitated by drugs |
Name 2 drugs which can cause aplastic anemia. | Chloramphenicol, Alkylating agents. |
Name 3 viral agents which can cause aplastic anemia. | Parvovirus B19, EBV, HIV |
Dx : Petechiae, infection, pallor. May follow acute hepatitis. | Aplastic anemia |
Dx : Pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration. | Aplastic anemia |
Aplastic crisis in sickle cell disease is associated with what? | Parvovirus B19 infection |
Dx : Autosplenectomy, increased risk of Strep. pneumonia infection, Salmonella osteomyelitis, painful vaso-occlusive crisis. | Sickle cell disease |
Name a pharmacological treatment of sickle cell disease. | Hydroxyurea (increase HbF) |
What is the genetic abnormality in Sickle cell anemia? | HbS mutation (substitution of glutamic acid with valine in beta chain) |
What will electrophoresis show in alpha-thalassemia with 3 gene deletions? | HbH |
What will electrophoresis show in alpha-thalassemia with 4 gene deletions? | Hb Bart |
What will electrophoresis show in Beta-thalassemia? | Decreased HbA, increased HbA2 and HbF |
What are the genetic problems which cause beta-thalassemia? | Mutations in splicing sites, and promoter sequences forming stop codon |
Cardiac failure in Beta-thalassemia patients is due to what? | Hemochromatosis. |
Give 3 examples of drugs which cause Drug-induced immune Hemolytic anemia. | Penicillin, quinidine, alpha-methyldopa |
Dx : Acute anemia seen with Mycoplasma pneumoniae infection, or infectious mononucleosis. | Cold (IgM) Autoimmune hemolytic anemia |
Dx : Chronic anemia seen in SLE or in CLL or with alpha methyldopa. | Warm (IgG) Autoimmune hemolytic anemia |
Dx : Splenomegaly, aplastic crisis (Parvovirus B19 infection), increased serum bilirubin, jaundice, increased reticulocytes | Hereditary spherocytosis |
Dx : Increased sensitivity of RBCs to lytic activity of complement | Paroxysmal nocturnal hemoglobinuria |
Dx : Impaired synthesis of GPI anchor or decay-accelerating factor in RBC membrane | Paroxysmal nocturnal hemoglobinuria |
What is the confirmatory test in hereditary spherocytosis? | Osmotic fragility test |
What is the screening test for Paroxysmal Nocturnal Hemoglobinuria? | Sucrose hemolysis test |
What is the confirmatory test for Paroxysmal Nocturnal Hemoglobinuria? | Acidified serum test (Ham test) |
Dx : Intravascular hemolysis seen in DIC, TTP/HUS, SLE, or malignant hypertension. | Microangiopathic hemolytic anemia |
Dx : Intravascular hemolysis seen in aortic stenosis | Macroangiopathic hemolytic anemia |
List the causes of DIC. | STOP Making New Thrombi Sepsis (gram-negative), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion |
Dx : Increased D-dimers, helmet-shaped cells and schistocytes on blood smear | DIC |
Dx : Deficiency of vWF-cleaving metalloproteinase | TTP |
Dx : E.coli (0157:H7) | HUS |
Dx : Bleeding disorder treated with desmopressin acetate and oral contraceptives | von Willebrand disease |
Dx : Abnormal ristocetin cofactor assay | von Willebrand disease |
Dx : Lacunar cells | Nodular sclerosing type of Hodgkin's lymphoma |
Dx : Fever, night sweats, weight loss, lymphadenopathy | Hodgkin's lymphoma |
Which type of Hodgkin's lymphoma is seen usually in women? | Nodular sclerosing type. |
Which type of Hodgkin's lymphoma is seen usually in older men? | Lymphocyte depleted type |
Which type of Hodgin's lymphoma is seen usually in males under 35 years old? | Lymphocyte predominant type. |
Dx : IgM spike, hyperviscosity symptoms | Waldenstrom's MMMacroglobinemia |
What are the symptoms of Multiple Myeloma? | CRAB hyperCalcemia Renal insufficiency Anemia Bone/Back pain |
What type of Non-Hodgkin's lymphoma is like CLL, with focal mass, and low grade? | Small lymphocytic lymphoma |
What type of non-Hodgkin's lymphoma is associated with t(14;18) and bcl-2 expression? | Follicular lymphoma |
What type of non-Hodgkin's lymphoma is associated with t(11;14), and CD5+? | Mantle cell lymphoma |
What type of non-Hodgkin's lymphoma commonly presents with ALL and mediastinal mass, and is a T-cell lymphoma? | Lymphoblastic lymphoma |
Dx : Starry sky appearance | Burkitt's lymphoma |
What is the genetic abnormality that caused Burkitt's lymphoma? | t(8;14) c-myc gene movess next to heavy-chain Ig gene (14) |
Dx : Leukemia with TdT+ | ALL |
Dx : Leukemia with smudge cell | CLL |
Dx : Leukemia which may spread to CNS and testes. | ALL |
Dx : Leukemia which cause warm autoimmune hemolytic anemia | CLL |
Dx : Leukemia which responds to imantinib | CML |
Dx : Leukemia which may transform to AML or ALL (blast crisis) | CML |
Dx : Leukemia which have DIC and respond to retinoic acid | M3 : Acute promyelocytic leukemia |
Dx : Birbeck granules on EM | Langerhans cell histiocytoses (histiocytosis X) |
Dx : Malignant histiocytosis, diffuse rash | Letterer-Siwe disease |
Dx : Malignant histiocytosis, rash on scalp and ear canal | Hand-Schuller-Christian disease |
Dx : Benign histiocytosis, pathologic fractures | Eosinophilic granuloma |
Dx : Thombotic events, increased histamine (red face, itchy after bathing), gout | Polycythemia vera |
Dx : Increased megakaryocytes, bleeding, splenomegaly | Essential thrombocytosis (produce increased platelet but they are abnormal) |
Dx : Myeloproliferative disorder with negative JAK2 mutation | CML |
Dx : Fibrotic obliteration of bone marrow | Myelofibrosis |
List the clinical pentad seen in TTP | Thrombocytopenia, Fever, MA hemolytic anemia, Renal, CNS |