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40 Cards in this Set
- Front
- Back
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New drugs SCA |
crizanlizumab voxelotor L-glutamine |
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Neonatal alloimmune thrombocytopenia |
IVIG corticosteroid whased maternal platlets |
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Bernard-Soulier syndrome platlet disorder adhesion receptor |
Drficiency of GP Ib complex (VWF receptor ) abnormal ristocetin test abnormal platlet size shape |
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Glanzmann thrombasthenia platetlet aggregation receptor |
GP IIb-IIIa deficiency
Normal count abnormal function related LAD typye 3 |
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VWD |
3 types type 1 partial type 3 complate type 2 dysprotenimia |
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Diamond blackfan anemia |
Macrocytic anemia without megablastic change Growth retardation Dx ⇧ erthrocyte adenosine deaminase |
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Kasabach-Merritt phenomenon |
Increased platlet destruction |
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Virchow's triad |
Endothelial injury Stasis of blood flow Hypercoagulability |
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Mean platlet volume |
5 fL |
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Dyskeratosis congenita DC Pancytopenia |
mutation telomere maintenance gene Dystrophy hair , nail , bone morrow failure , skin pigmentation , oral leukoplakia , dental changes , pulmonary fibrosis , cancer risk |
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Pearson syndrome pancytopenia |
Mitochondrial disorder Bone morrow failure Excrorine pancreatic insufficiency CNS , mascular imparmant |
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Shwachman-Diamond syndrome isochromsome 7q |
Neutropenia 90%, anemia 46%, thrombocytopenia 42%, and pancytopenia 21% Pancytopenia pancreatic insufficiency Skeletal abnormalities |
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Cord blood sample |
-Hemoglobin of 16.0 gm/dl, -WBCs of 18,000/cmm, -neutrophils of 60% -reticulocytes of 5% |
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30% |
Thirty percent of children who are iron deficient develop iron deficiency anemia |
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Basophilic stippling |
lead poisoning Thalassemia myelodysplasia |
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Mentzer index ( thalassemia ) |
MCV divided by RBCs count > 12.5 thalassemia |
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Anemia |
Low serum iron, low serum ferritin, high iron binding capacity, reduced marrow iron stores, decreased marrow sideroblasts and high free erythrocyte protoporphyrin, normal electrophoresis and high red cell distribution width |
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Hepcidin |
liver protein plays role in iron homeostasis |
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Severe aplastic anemia |
absolute reticulocyte count less than 20,000/mm3, and bone marrow cellularity on biopsy specimen less than 25% of normal |
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Thrombin time |
Fibrinogen to fibrin |
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Transfusion |
Packed red blood cell; rise of Hb by 2-3 gm/dl, platelets concentrate; rise of platelets by 30.000 50.000/ml, Cryoprecipitate rise fibrinogen 50-100 recombinant factor VIII; rise of factor VIII by 2%/unit/kg, and recombinant factor IX; rise of factor IX by 0.5%/unit/kg |
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Cryoprecipitate contain |
Factors VIII and XIII, VWF and fibrinogen |
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Extremly high RDW |
sideroblastic anemia |
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PNH |
Dx flow cytometry anti CD-55 & anti CD-59 |
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Factor XIII deficiency |
Tx cryoprecipitate |
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Gamma irradiation |
graft versus host disease |
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High PTT |
factor XII, prekallikrein, and high molecular weight kininogen |
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Factor VII deficiency |
Spontinous intracaranial deficiency |
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Factor V leiden mutation |
... |
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Reticulocyte index active erythropoiesis of the bone marrow |
Reticulocyte index = reticulocyte count× (observed hematocrit/normal hematocrit |
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Factor XIII deficiency |
clot solubility test |
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IDA vs AOCD |
Soluble transferring receptor (sTfR) is a diagnostic test used to distinguish ACD from iron-deficiency anemia (IDA); sTfR levels are high in IDA and normal in ACD |
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Swiss cheese |
Congenital dyserythropoietic anemia |
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Tangier disease |
, familial deficiency of high-density lipoproteins(HDLs) is a rare recessive disorder that results from mutationsin the cholesterol and phospholipid transport protein ABCA1, leading to perturbations of cellular cholesterol transport, and resulting in the accumulation of cholesterol esters in many tissues. Hematologic manifestations include a mild to moderate stomatocytic hemolytic anemia and thrombocytopenia. Affected patients can also have large orange tonsils, hepatosplenomegaly, lymphadenopathy, cloudy corneas, peripheralneuropathy, and premature atherosclerosis |
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Revesz syndrome |
has many of the features of DC and presents in early childhood in addition to Bilateral exudative retinopathy is required to establish a diagnosis. Patients may also have intracranial calcifications, IUGR, developmental delay, and bone marrow failure |
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MYH9-related thrombocytopeni |
acomprises a number of diverse hereditary thrombocytopenia syndromes (e.g., Sebastian, Epstein, May-Hegglin, Fechtner) characterized by autosomal dominant macrothrombocytopenia, neutrophil inclusion bodies, and a variety of physical anomalies, including sensorineural deafness, renal disease, and eye disease |
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splenic hypofunction |
Pitted erthrocyte |
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Castleman disease |
Human herpes virus 8 |
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TAFRO syndrome stands for |
Thrombocytopenia, Anasarca, Fever, Reticulum fibrosis, and Organomegaly |
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POEMS syndrome stands for |
Polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin lesions |
