Hematology Flashcards
The other major natural cobalamin that is the form in human plasma and in cell cytoplasm.
methylcobalamin,
*It is now thought that metformin lowers serum vitamin B12 level by lowering thelevel of
TC I
The goal of therapy in IDA is to provide shoes of at least
0.5 to 1gram of IRON
sustained tx 6-12months needed to achieve this
True or false:
DIC is diagnosed in almost one-half of pregnant women with abruptio placentae or with amniotic fluid embolism.
True
is a severe form of DIC resulting from thrombosis Of extensive areas of the skin; it affects predominantly young children following viral or bacterial infection, particularly those with inherited or acquired hypercoagulability due to deficiencies of the components of the protein C pathway
Purpura fulminans
The central mechanism of DIC is the
uncontrolled generation of thrombin by exposure of the blood to pathologic levels of tissue factor
The most common inherited factor deficiencies are the
hemophilias
An isolated abnormal prothrombin time (PT) suggests
FVII deficiency 7
prolonged activated partial thromboplastin time (aPTT) indicates most commonly
hemophilia or FXI deficiency
The prolongation of both PT and aPTT suggests deficiency of
FV, FX, FII, or fibrinogen
What is the mainstay of treatment for TTP?
Plasma exchange
What is the PENTAD for TTP?
Microangiopathic hemolytic anemia Thrombocytopenic purpura Neurological abnormalities Fever Renal disease
WHO considers how much % of blast to distinguish AML from MDS?
20% last in the marrow
What clinical finding differentiates PCV from other causes of ERYTHROCYTOSIS?
Skin itching on contact with water
Aquagenic pruritus
In most PV Patients, once an iron-deficient state is achieved , PHLEBOTOMY is usually only required at
3months intervals.
Iron chelating agents for hemosiderosis
Deferoxamine (IV PARENTERAL)
Desferasirox (oral)
The gold standard diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)
Flow cytometry
Preferred treatment for PNH
ECULIZUMAB
Pure red cell a plasma is compatible with long term survival with supportive care alone such as
A combination of ERYTHROCYTE TRANSFUSIONS and IRON chelation.
For persistent B19 parvovirus infection, almost all patients respond to
IV Immunoglobulin therapy
The majority of patients with IDIOPATHIC PRCA respond favorably to IMMUNOSUPPRESSION such as
Glucocorticoids Cyclosporine ATG Azathioprine Cyclophosphamide
Mutation of this gene plays a central role in the pathogenesis of Polycythemia Vera as well as other myeloproliferative neoplasias
JAK 2
Is a distinctive manifestation characterized by chest pain, tachypnea , fever, cough and arterial oxygen desaturation. It can mimic pneumonia , PE, bone marrow infarction and embolism, MI or in situ lung infarction.
Acute chest syndrome
What drug should be considered for patients experiencing repeated episodes of acute chest syndrome or with more than 3 crises per year requiring hospitalization
Hydroxyurea
is the major protease enzyme of the fibrinolytic system
plasmin
The most sensitive test for DIC is the
FDP level
Fibrin degradation product level
The skin and mucous membranes may be pale if thehemoglobin is
<80–100 g/L (8–10 g/dL)
(; an indirect measure of serum transferrin)
TIBC
Marked alterations in the red cell indices usually reflect disorders of
maturation or iron deficiency
hyperviscosity and thrombosis (both venous and arterial), because the blood viscosity increases logarithmically at hematocrits >
55%
True or false
At least 75% of all cases of anemiaare hypoproliferative in nature
True
Suppression of EPO BY reduced tissue needs-for OXYGEN from metabolic disease such as
hypothyroidism
Patients with the anemia of acute or chronic inflammation show a distinctive pattern of serum iron (),TIBC (), percent transferrin saturation (), and serumferritin ()
serum iron (low),TIBC (normal or low), percent transferrin saturation (low), and serumferritin (normal or high)
These changes in iron values are brought about by_______, the iron regulatory hormone that is produced bythe liver and is increased in inflammation
hepcidin
distinct pattern of results is noted in
mild to moderate iron deficiency
low serumiron, high TIBC, low percent transferrin saturation, low serum ferritin)
The diagnosis of β-thalassemia major is readily made during childhood on the basis of severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis:
hepatosplenomegaly,
profound microcytosis, a characteristic blood smear,
and elevatedlevels of HbF, HbA2, or both
folic acid should be given as a supplement before and throughout pregnancy to prevent megaloblasticanemia and reduce the incidence of NTDs, even in countries withfortification of the diet
400 μg daily
In women who have had a previous fetus with an NTD,what is the recommended dose when pregnancy is contemplated and throughout the subsequent pregnancy.
5 mg daily (5000 mcg)
True or false:
Cobalamin should be given routinely to all patients whohave had a total gastrectomy or ileal resection
True
Dosage for cobalamin therapy
Replenishment of body stores should be complete with
six1000-μg IM injections of hydroxocobalamin given at 3- to 7-dayintervals
Causes of Cobalamin Deficiency Sufficiently Severe toCause Megaloblastic Anemia
Pernicious anemia Congenital absence of intrinsic factor Total or partial gastrectomy Intestinal stagnant loop syndrome: jejunaldiverticulosis, ileocolic fistula, anatomic blind loop,intestinal stricture, Ileal resection and Crohn’s disease Selective malabsorption with proteinuria Tropical sprue Transcobalamin II deficiency Fish tapeworm
characterized by microangiopathic HA with presence of Fragmented erythrocytes in the peripheral blood smear, thrombocytopenia (usually mild), and acute renal failure.
Familial (Atypical) Hemolytic-Uremic Syndrome (aHUS
warm antibody AIHA
WARM, MOSTLY IgG, OPTIMAL-TEMPERATURE 37°C;OR MIXED
SLE
CLL
Majority: currently most common culprit drugs are cefotetan,ceftriaxone, piperacillin
Classification of Acquired Immune Hemolytic Anemias: COLD, MOSTLY IgM, OPTIMAL-TEMPERATURE 4°C–30°C
EBV
CMV
Mycoplasma infection:paroxysmal cold hemoglobinuria
Waldenstróm’s disease Lymphoma
This triad makes PNH Paroxysmal Nocturnal Hemoglobinuria
i) hemolysis
ii) pancytopenia
iii) a distinct tendency To venous thrombosis
The definitive diagnosis of PNH must be based on the demonstration that a substantial proportion of the patient’s red cells have an increased susceptibility to
complement (C)
replacement of the bone marrow by fat is apparent in the morphology of the biopsy specimen
and Magnetic resonance imaging (MRI) of the spine
APLASTIC ANEMIA
Caféau lait spots and short stature suggest;
Fanconi anemia
characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow + occasional THYMOMA
(thrombocytopenia with amegakaryocytosis and neutropenia without marrow myeloid cells in agranulocytosis)
Pure red cell aplasia
are a heterogeneous group of hematologic disorders broadlycharacterized by both (1) cytopenias due to bone marrow failure and(2) a high risk of development of AML
Myelodysplasia
Childrenwith Down syndrome are susceptible to
MDS
What is the only management for MDS
Only hematopoietic stem cell transplantation offers cure of MDS
Which hematologic abnormality can be found in anemia of liver disease
Shortened red cell survival
What Level of prothrombin time is a good indicator of the severity of clotting factor consumption?
> 1.5 x the normal
Which agent is the most consistently associated with aplastic anemia?
Hydantoins
What type of diarrheal infection is associated with HUS
E. coli
