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FA Hemato Patho

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
Created by: lemontea88



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