blood transfusion reaction whose onset is over 24hrs(1 -2weeks)
delayed hemolytic transfusion reaction(DHTR)
→
Anamnestic response to a foreign antigen on donor RBCs (most commonly Rh or ☆other minor blood group antigens) previously encountered by recipient hematologyUWorld Mar 7, 2020, 2:23 AM HirotoShishido
erythropoiesis stimulating agent
indication, adverse effectsanemia of chronic disease
what drug is responsiblesideroblastic anemia
☆ringed sideroblast are seen in bone marrow with Prussian blue stain
☆isoniazid, linezolid, chloramphenicol
what triggers vaso occlusive crises in sickle cell anemia
oxygen affinity of sickle celloxygen unloading
☆↓ oxygen affinity due to stabilizing effects of polymerization on the deoxygenated form hematologyUWorld Jan 14, 2020, 8:02 AM HirotoShishido
warm autoimmune hemolytic anemia(AIHA)
associated diseaseSLE, CLL
(and with certain drugs (eg)α-methyldopa) hematology Jan 2, 2020, 12:12 AM HirotoShishido
sickle cell anemia
treatmenthydroxyurea(treatment for frequent and severe pain)
hydroxyurea ☆increases fetal hemoglobin production (HbF is unaffected by sickle cell mutation because it's not compoted of beta chains(ααγγ)), which lowers the risks of RBC sickling and sickling-related complications pharmacologyhematologyUWorld Dec 19, 2019, 6:22 AM HirotoShishido
PictureHeinz bodies
G6PD deficiency
Hb precipitation→subsequent phagocytic damage to RBC membrane→bite cells hematology Dec 19, 2019, 2:45 AM HirotoShishido
acute chest syndrome
symptoms(painful crisis in sickle cell anemia)
pathogenesis/ causes/ manifestation/ first line chemotherapy for myelodysplastic syndrome (MDS)Stem cell disorders involving ineffective hematopoiesis → defects in cell maturation of nonlymphoid lineages.
Caused by de novo mutations or environmental exposure (eg, radiation, benzene, chemotherapy). Risk of transformation to AML.
ringed sideroblast,
Pseudo-Pelger-Huet anomaly- neutrophils with bilobed (“duet”) nuclei. Typically seen after chemotherapy.
☆pitechiae, weakness, recurrent infections
(splenomegaly is uncommon)
characteristics of T-cell acute lymphoblastic leukemia(ALL)accounts for 15-17% of all ALL
T-ALLs tend to manifest as thymic lymphomas(anterior mediastinal mass) ☆in late childhood through young adulthood
(presenting as SVC-like syndrome, ☆dysphagia, )
positive for terminal deoxynucleotidyl transferase(TdT+)(marker for pre-T and pre-B cells, both for B and T ALLs)
t(12;21) has better dianosis hematologyUWorld Dec 13, 2019, 11:34 PM HirotoShishido
paroxysmal nocturnal hemoglobinuria (PNH)
labs, complications, treatment☆CD55/59➖RBCs on flow cytometry
Chronic hemolysis with breakdown of iron-containing erythrocytes can also lead to iron deposition in the kidney (☆hemosiderosis), which can interfere with proximal tubule function and cause interstitial scarring and cortical infarcts. The hemosiderosis combined with microvascular thrombosis can increase the risk of CKD.
+ thrombosis at atypical site(hepatic, portal, cerebral), pancytopenia
the treatment for pregnant women with DVT at less than 37 weeks gestationlow molecular weight heparin (LMWH), such as ☆enoxaparin, is preferred over unfractionated heparin pharmacologyOBGYhematology Dec 6, 2019, 8:52 PM HirotoShishido
β-thalassemia major
treatment and adverse effect(β chain is absent)
Causes marrow fibrosis → ☆dry tap on aspiration.
☆LUQ pain, massive splenomegaly, pancytopenia
cladribine(antimetabolites, ☆ADA inhibitor)
☆ADA converts adenosine/deoxyadenosine into inosine/deoxyinosine
→ inhibition of ADA activates alternate pathyway, leading to lymphocyte apoptosis hematologyUWorld Nov 23, 2019, 11:32 PM HirotoShishido
