NEW hematology (copy)

what are the pros and cons of using umbilical cord stem cells for transplantation?

pro: it is the fastest method out of all 3

con: it has a higher incidence go engraftment failure

what is the treatment for TTP?

plasma exchange therapy

what is polyclonal gammanopathy?

overproduction of more than one class of protein (alb, alpha, beta, gamma)

what are the appropriate treatments for multuple myeloma?

-mib drugs (bortezomib) and thalidomides

explain the pathophysiology of DIC

when the body has a severe infection or trauma (pregnancy complications), there is an increase in damaged endothelium.

when there is a gap in the endothelial cell layer, leukocytes release tissue factor , which then stimulates clot formation.

clots begin to form around the body, which lead to hypoxia, tissue damage, MAHA, and a consumption of platelets and coagulation factors.

this leads to abnormal bleeding and hypotension.

explain the pathophysiology of TTP

TTP is caused by a severe deficiency of ADAMTS15 enzyme, which breaks down the von Willebrand factor (clotting protein). it is most commonly caused by immunodeficiency.

there is an increase in clot formation and platelets are consumed (not coagulation factors)

symptoms arise dramatically and include fever, nausea, kidney failure, and neurological abnormalities.

explain the pathophysiology of ITP

number of platelets decrease due to an autoimmune reaction. coagulation factors are intact.

children can get acute ITP, whereas adults get chronic ITP

decreased platelets, increased megakaryocytes, anti-platelet antibodies are positive.

what are the symptoms of primary macroglobinemia?

easy bruising, fatigue, weight loss, vision changes (sausage appearance in fundoscopy)

hyperviscosity syndrome

decreased blood flow in the peripherals. (raynaud’s phenomenon)

lymph node swelling

hepatosplenomegaly

differentiate between aplastic anemia and pure red cell aplasia

aplastic anemia affects all 3 cell types (pancytopenia) bone marrow aspiration shows fatty marrow

pure red cell aplasia affects only RBCs., (erythropenia) bone marrow aspiration shows low erythroblasts in bone marrow.

what are the PT and APTT for DIC?

both are prolonged (because all coagulation factors are consumed)

what is the treatment of DIC?

treat the underlying cause

fresh frozen plasma

IV unfractioned heparin

what are the blood test findings of anemia of chronic disease?

decreased serum Fe

decreased transferrin

decreased total iron binding capacity (TIBC)

increased serum ferritin

what disease is developed after polycythemia vera?

myelofibrosis

lab findings of polycythemia vera?

increased Hb, increased Hct, incrased WBC, increased platelets

what diseases are indicated when BMA is dry tap?

metastatic carcinoma

acute leukemia

myelofibrosis

what is the most common immunodeficiency that causes anaphylaxis in blood transfusion?

selective IgA deficiency

what type of anti-coagulant drug that causes psudothrombocytopenia?

EDTA

what are the 3 steps of stem cell transplant

conditioning (with high-dose chemo) to reduce tumor cels

stem cell infusion

engraftment

what drug is most commonly used for conditioning in stem cell transplant?

cyclophosphamide

what drug is used as prophylaxis for GVHD?

methotrexate and FK or CsA

how is GVHD treated?

systemic steroid therapy

what are the signs and symptoms of pernicious anemia?

weakness, fatigue, mouth ulcers, glossitis, weight loss, jaundice, paresthesia.

why does primary myelofibrosis cause splenomegaly?

because the marrow has fibrosis so it leads to extramedullary hematopoeisis

what is the treatment of myelofibrosis?

splenectomy, steroids, low dose thalidomide, ruxolitinib, allogenic bone marrow transplant

what is HUS?

disease caused by an E. coli infection. most commonly effects children.

causes diarrhea, MAHA, acute renal failure, thrombocytopenia

what type of lymphocytes have surface immunoglobulins?

mature B cells

what is the surface immunoglobulin in naive B cells?

IgM and IgD

what is the surface immunoglobulin in B cells after a class switch?

IgG, IgA, IgM

what are the 3 negative regulators of coagulation pathway

antithrombin, thrombomodulin, protein C

how many mg of iron is contained in 1g of hemoglobin?

3.4mg iron

how many mg of iron is contained in 1ml of blood?

0.5mg iron

what type of hemolysis occurs in PNH (Paroxysmal nocturnal hemoglobinuria)?

complement-mediated intravascular hemolysis

what mutation is occuring in PNH (Paroxysmal nocturnal hemoglobinuria)?

PIG-A

what are the 3 treatments of ITP?

TPO receptor agonist, splenectomy, steroids

what mutations are found in essential thrombocytosis?

JAK2, CALR, MPL

what are the clinical signs of essential thrombocytosis?

persistently elevated platelet count

megakaryocyte hyperplasia

splenomegaly

thrombotic and hemorrhagic episodes

mutations in JAK2, CALR, or MPL gene

what are the treatments for essential thrombocytosis?

aspirin, anagrelide, warfarin (to prevent thrombus), plateletpheresis (for emergencies), cytoreductive therapy (for high-risk patients)

which is more abundant, T cells or B cells?

T cells

what diseases increase the production of M protein?

multiple myeloma, al amyloidosis

what is the treatment fo AIHA?

corticosteroids, splenectomy if necessary

list diseases causing splenomegaly

chronic leukemia

hemolytic anemia

myelofibrosis

TTP

list 4 types of B-cell lymphoma

follicular lymphoma

mantle cell lymphoma

burkitt lymphoma

extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue

what is the difference between TACO (transfusion-related circulatory overload) and TRALI (transfusion-related acute lung injury)?

With both, patients present with respiratory distress due to acute onset pulmonary edema.

With TRALI, patients also often have hypotension and fever, and can have transient leukopenia. With TACO, one would typically expect hypertension and a lack of fever and leukopenia.

what is the treatment for aplastic anemia?

cyclosporine A

what drug is used to treat APL when it has relapsed?

arsenic trioxide (ATO)

what method is used for imaging modality for initial staging of diffuse B-cell lymphoma?

FDG-PET/CT

what is the treatment for hereditary spherocytosis?

0-5 years old: folic acid supplements

5 years and older: splenectomy

adults: splenectomy and cholecystectomy

describe the morphology of tumor cells of B-cell chronic lymphoid leukemia?

small mature lymphocytes

what type of anemia is associated as a complication of B-cell CLL?

autoimmune hemolytic anemia

what is richter syndrome?

an aggressive histologic transformation of chronic lymphocytic leukemia (CLL), most commonly to diffuse large B-cell lymphoma (DLBCL). Outcomes are generally poor.

what are the EPO levels like in pylecythemia vera?

low, due to negative feedback

what diseases are associated with paroxysmal nocturnal hemoglobinuria?

aplastic anemia, myelodysplastic syndrome, AML

what are the clinical signs and symptoms of paroxysmal nocturnal hemoglobinuria?

dark-color urine that comes and goes

abdominal and pack pain

headache and shortness of breath

coombs test negative hemolysis

bone marrow may be aplastic, hypoplastic, or dysplastic

what are the 2 methods to prevent severe GVHD from haploidentical transplant?

ATG (anti-thymocyte globulin) before the transplant

high-dose cyclophosphamide after the transplant

what is ATG?

horse or rabbit-derived antibodies against human T cells (impairs human T cell function)

what complication is caused by alloreactive T-cells in haploidentical transplant?

haplo-immunostorm fever

when does transfusion-associated GVHD occur after a transfusion?

8-10 days after

what is the mortality of transfusion-associated GVHD?

over 90%

what are the symptoms of transfusion-associated GVHD?

fever

rash

diarrhea

liver dysfunction

severe pancytopenia

what are the risk factors of transfusion-associated GVHD?

whole blood transfusion

elderly patients

women

cancer patients

history of thoracotomy

when the donor is a close relative

what are the 3 ways to prevent transfusion-associated GVHD?

gamma-irradication (inhibition of donor lymphocyte proliferation) of blood products

use a leukocyte removing filter

don’t transfuse blood of close family members or relatives.

what is CAR-T cell?

(chimeric antigen receptor - t cell)

a T cell that is introduced an artificially designed CAR to target certain cells.

what was the first CAR-T treatment in Japan and what diseases was it used for?

Tisagenlecleucel (kimuria)

used for B cell ALL relapse, CD19+ diffuse large B cell lymphoma

What antigen is used for the target of CAR-T cell therapy for ALL, diffuse large B cell lymphoma, and follicular lymphoma?

CD19

What antigen is used for the target of CAR-T cell therapy for hodgkin’s lymphoma?

CD30

What antigen is used for the target of CAR-T cell therapy for multiple myeloma?

CD38 and BCMA

What antigen is used for the target of CAR-T cell therapy for AML?

CD33 and CD123

what are the symptoms of CLL?

usually asymptomatic, as long as there are no complications

what does the flowcytometry show in CLL?

CD5+, CD19+, CD20dull, CD23+, surface Ig+

what are the complications of CLL?

Richter’s syndrome

frequent infections

AIHA

ITP

what are the 2 examinations (additional to blood work) that should be done when CLL is suspected?

southern blotting of immunoglobulin rearrangement

flowcytometry analysis

what are the treatments for CLL?

purine analogue (fludarabine)

alkylaring agents (chlorambucil and cyclophosphamide)

anti-CD20 antibodies (-mab drugs, such as rituximab)

what clinical signs are unique to iron deficiency anemia?

painless glossitis

spoon nails

unusual dietary cravings, such as pagophagia

how to calculate MCH?

(hemolgobin / RBC) x 10

how to calculate MCHC?

(hemolgobin / Hematocrit) x 100

how to calculate MCV?

(hematocrit / RBC) x 10

what is hepcidin?

controls the transportation of iron to blood plasma.

what are the hepcidin level changes in IDA and ACD?

IDA - decreased hepcidin in blood

ACD - increased hepcidin in blood (high hepcidin levels inhibit the release of iron from transferrin into blood)

how is each type of ATL managed?

chronic and smoldering type - observation

acute and lymphoma type - chemotherapy or stem cell transplant. (prognosis is poor)

what molecular targetting drug is used for ATL?

mogamulizumab (anti CCR4 antibody)

what are the 2 factors that inhibit proliferation of activated T cells, resulting in tumor cell death?

PD-1 and CTLA-4

what is the surface antigen of plasma cells?

CD38

what glycoprotein binds to von willebrand factor?

GP lb

what glycoprotein binds to fibrinogen?

GP IIb and IIIa

what does FDP consist of?

fibrin and fibrinogen degradation products?

what is TAT used for?

reflects the activation of coagulation reactions

what is the function of ATRA?

differentiation function

what are the two types of cytotoxicity in rituximab?

ADCC and CDC

which antigens and their ligands do immune checkpoint inhibitor such as nivolumab block?

PD-1

what disease is suspected when peripheral blood smear shows teardrop RBCs?

myelofibrosis

what is the cause of primary erythrocytosis?

polycythemia vera

what is the cause of secondary erythrocytosis?

sleep apnea syndrome

diseases of heart and lung

residing in high altitude areas

what is the cause of relative erythrocytosis?

dehydration

what are the treatments for polycythemia vera?

phlebotomy

low-dose aspirin

hydroxycarbamide (for high risk)

what is the pharmacological effect of anagrelide?

phosphodiesterase 3 inhibitor

what are the adverse effects of anagrelide?

palpitation and headache

what is the most dominant subtype of lymphoma?

diffuse large b cell lymphoma

in AL amyloidosis, what test is used to assess hematological response?

free light chain test

what is the chemotherapy treatment regimen for B-cell lymphoma?

R-CHOP (rituximab, cyclophosphamide, hydroxydaunomycin, oncovin, prednidolone)

what is the treatment regimen for hodgkin’s lymphoma?

ABVD