treatment of aplastic anemia• immunosuppressive therapy (for idiopathic aplastic anemia)
■ horse or rabbit ☆anti-thymocyte globulin: 40-50% of patients respond
■ + cyclosporine (for improved response and survival)
• allogeneic bone marrow transplant
→ for younger patient(=<40yo)
• growth factors: e.g. Eltrombopag (TPO receptor agonist), G-CSF, and EPO noteffective hematology国試過去3回 Oct 16, 2020, 2:18 AM HirotoShishido
Transient Abnormal MyelopoiesisDown症の10%以上に合併する「一過性骨髄異常増殖症(TAM)」
TAMはDown症の約10%に合併するとされ,末梢血中で白血病様の巨核芽球が増加することで肝脾腫や血小板減少をきたす疾患である.重症例では肝機能障害や血小板減少,DICなどが起こり,近年の報告では臓器障害で20〜30%の患者が死亡しているとされている.自然治癒が期待できるため経過観察が基本であるが,重症ならば積極的な治療介入が必要とされること,治癒例でも20〜30%がM7を発症するためフォローアップが重要である pediatricshematology国試過去3回 Oct 13, 2020, 2:34 AM HirotoShishido
心房細動への抗凝固療法弁膜症性心房細動における抗凝固療法の適応はワルファリンのみである一方,非弁膜症性心房細動では,ワルファリン以外にも直接経口抗凝固薬〔(DOAC)直接トロンビン阻害薬:ダビガトラン,第Xa因子阻害薬:リバーロキサバン,アピキサバン,エドキサバン〕が適応となる cardiologyhematology国試過去3回 Oct 12, 2020, 2:43 AM HirotoShishido
easy bruising and hemarthrosis accompanied with Crohn disease☆bile acid malabsorption
→ fat malabsorption
→ vitamine K deficiency gastrointestinalhematologyUWorld Jul 9, 2020, 4:02 AM HirotoShishido
type of Hodgkin lymphoma・Nodular sclerosis
Most common
・Lymphocyte rich
Best prognosis
・Mixed cellularity
Eosinophilia, seen in immunocompromised patients
・Lymphocyte depleted
Seen in immunocompromised patients hematology Jul 6, 2020, 1:26 AM HirotoShishido
erythropoietin in polycythemia vera(PV)
EPO decreases due to negative feedback suppressing renal EPO production hematology Jul 4, 2020, 12:10 AM HirotoShishido
prothrombin gene mutationMutation in 3′ untranslated region →↑production of prothrombin → ↑plasma levels and venous clots. hematology Jul 3, 2020, 10:29 AM HirotoShishido
affinity of hemoglobin A and F to oxygenFetal hemoglobin (hemoglobin F) is synthesized primarily during fetal development (-8 weeks until term) and consists of the usual 2 alpha chains with 2 gamma chains in place of beta chains. The gamma chains do not bind effectively to 2,3-BPG due to replacement of a ☆histidine residue with serine. hematologyUWorld Jul 3, 2020, 9:07 AM HirotoShishido
PictureHowell- Jolly bodies
(round, and bigger than pappenheimer)
Functional hyposplenia(eg sickle cell disease), asplenia
Basophilic ★RBC nuclear remnants (do not contain iron)
Usually removed by splenic macrophages
Basophilic stippling (many)
Sideroblastic anemias, thalassemias
Basophilic (★)ribosomal precipitates (do not contain iron)
characteristics of plasma cells☆"Clock-face" chromatin distribution and eccentric nucleus(perinuclear paleness), abundant RER, and well-developed Golgi apparatus.
bone marrow sample with >10% plasma cells strongly suggest multiple myeloma (MM) hematologyUWorld Jul 1, 2020, 9:17 AM HirotoShishido
what is extramedullary hematopoiesis suggestive of☆chronic hemolysis(eg β-thalassemia)
+MF
+toxoplasma, cytomegarovirus, rubella infection(→blueberry muffin rash= dermal extramedullary hematopoiesis) hematologyUWorld Jun 28, 2020, 1:58 AM HirotoShishido
PictureThe reticulocyte is slightly larger (10 microns) than the mature erythrocyte. Reticulocytes appear basophilic, reticular(net-like) of ☆ribosomal RNA on the Wright-Giemsa-stained smear (A)
brilliant crystal blue, is used to stain reticulocytes (indicated by the arrows in Image B) for an actual count (B) hematologyUWorld Jun 25, 2020, 10:26 PM HirotoShishido
myoglobin vs hemoglobinmyoglobin→ monomeric protein, primary oxygen storing protein in skeletal and cardiac muscle tissue
p50= 2.8mmHg
hemoglobin→ tetramer cosisting of 2 α and 2 β chains
after binding to one oxygen molecule, the oxygen affinity of other heme molecules increase
p50= 26mmHg hematologyUWorld Jun 25, 2020, 10:02 PM HirotoShishido
clinical use/ side effects of erythropoiesis-stimulating agents(ESAs)(eg. darbepoetin alpha, erythropoietin)
anemia of chronic kidney disease
thromboembolic events(eg stroke, vascular graft thrombosis) due to increased blood viscosity, ☆ worsening hypertension due to activation of erythropoietin receptors on vascular endothelial and smooth muscle cells hematologypharmacologyUWorld Jun 17, 2020, 5:36 AM HirotoShishido
leukemoid reactionbenign leukocytosis (>50,000/mm3) that occurs in response to an underlying condition such as severe infection/hemorrhage, solid tumors, or acute
hemolysis
The bone marrow can be normal or hypercellular and responds with
increased bands and ☆early mature neutrophilprecursors (eg, myelocytes),in
contrast to the increased immature cells (eg, promyelocytes, myeloblasts) seen in acute leukemia.
☆↑ALP(vs CML), peripheral smear can show Dohle bodies= ☆basophilic oval inclusions in mature neutrophi... hematologyUWorld Jun 16, 2020, 8:44 AM HirotoShishido