Hem/onc Tx And Tests Flashcards by lauren Poe

What is the tx for mild hemophilia?

DdAVP

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How do you diagnose a bleeding disorder?

Get PT, PTT, TT, fibrinogen and bleeding time
Conduct a mixing study
Obtain factor assays

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What is the tx for bleeding episodes due to hemophilia?

Transfusion of clotting factors or cryoprecipitate to at least 40% normal concentration

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How do you diagnose vWF disease?

Labs : prolonged PTT and bleeding time

Ristocetin cofactor assay is decreased

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How do you treat vWF?

DDAVP

Tx menorrhagia with OCPs

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What are diagnostic criteria for diagnosing a patient with a hereditary hypercoaguable state?

Must have 2 abnormal values while asx and untreated with 2 abnormal values in 2 other family members

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How do you treat a DVT or PE?

Give heparin or LMWH
3-6 months of warfarin for first event
6-12 months for the second event
Lifelong anticoag if it happens 3 times

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What is the best means of preventing a DVT or PE in a patient who can’t be anticoagulated?

An IVC filter

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How so you diagnose DIC?

PT, PTT, d-dimer, fibrin split products are increased

Fibrinogen level and ALL clotting factors are decreased

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What is the tx for DIC.

RBC transfusion
Platelet transfusion
Shock management

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What is the tx for HIT.

Stop heparin

Start lepirudin or argatroban

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What is the tx for TTP?

Plasma replacement
Plasmapheresis
Steroids to decreases micro thrombus formation

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What is CI in TTP?

Platelet transfusion - can make it worse!

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How would you diagnose ITP?

Bone marrow aspirate would show increased megakaryocytes

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When do you tx ITP?

When patients have symptomatic bleeding (usually when platelets are below 20,000)

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What is the tx of sx ITP?

Corticosteroids
High dose gamma globulin
Splenectomy (consider if cannot be tapered off of corticosteroids after 3-6 months)

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What are the second line therapies for ITP?

Rituximab

Anti-D immunoglobulin

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What test distinguishes between an absorption problem and a dietary deficiency of B12?

Schilling test

Give B12 IM and then give radiolabelled B12 - if shows up in the urine the problem is a dietary deficiency

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What lab values are consistent with hemolytic anemia?

High LDH, elevated indirect bilirubin and decreased haptoglobin levels
High reticule yet count

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What is used to detect autoimmune hemolytic anemia?

A Coomb’s test

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How do you diagnose aplastic anemia?

CBC and clinical

Bone marrow bx : hypocellularity and fat

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What is the tx of aplastic anemia?

Blood transfusion
Stem cell transplantation
Cyclosporine A and antithymocyte globulin for immune suppression and to prevent autoimmune destruction of the marrow

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What is the gold standard for diagnosing sickle cell?

Hemoglobin electrophoresis

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How else is sickle cell diagnosed?

Peripheral blood smear

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What is the tx for hereditary spherocytosis?

Splenectomy

How do you diagnose hereditary spherocytosis?

CBC shows low MCV and high MHCH And high RDW Osmotic fragility test= pos See sphere RBCS and Howell-jolly bodies on peripheral smear

How do you diagnose G6PD?

Do enzyme assay right after an episode | Peripheral smear shows : Heinz bodies and bite cells

What is the tx for PNH?

Eculizumab

What is tx for autoimmune hemolytic anemia?

IVIG Splenectomy Steroids

What is the tx of SCD?

Hydroxyurea Chronic folate supplement Pneumococcal vaccination And penicillin BID when patient is less than 5 (MCC of death in children is pneumococcal sepsis)

What is the tx of veno-occlusive crises in SCD?

Oxygen IVF Pain control Antibiotics if infection was the trigger

How do you keep VOC from progressing to acute chest syndrome?

IVF and incentive spirometry | Keep sickling to less than 40% with simple transfusion or exchange transfusion in ICU

What is the chronic tx for SCD when the fail hydroxurea therapy?

Chronic transfusions

How are thalassemia s diagnosed?

Hemoglobin electrophoresis | DNA studies

How do you treat B-thal major?

Chronic transfusion BM transplant Need deferoxamine too to prevent iron overload

How do you great hemoglobin H disease?

Chronic blood transfusion + iron chelation

How do you diagnose PCV?

CBC with ABGs Levels of EPO JAK2 mutation test

What is the tx of PCV?

Phlebotomy, keep Hct under 45% ASA (because prone to thrombotic events) Hydroxyurea or INF (cytoreductive drugs)

How do you diagnose porphyria?

Elevated blood, urine and stool porphyrins | Enzyme assays

What is the tx of porphyria?

Heme Glucose (to decreased heme synthesis) INF alpha if have Hep C, hydroxychloroquine, phlebotomy

How do you diagnose leukemia?

Greater than 20-30% Blasts on smear | Bone marrow aspirate

How do you treat AML and ALL?

``` Chemo Antibiotics Transfusion Colony-stimulating factor Bone marrow transplant ```

What should be started to prevent tumor lysis syndrome?

Allopurinol

How do you treat leukostasis syndrome?

Hydroxurea and leukapheresis (to decrease WBC)

How do you diagnose CLL?

Flow cytometry demonstrating the presence of CD5, 20 and 21 | CBC shows lymphocytosis ( greater than 5,000) and smudge cells

When do you treat CLL?

When the patient becomes symptomatic

How do you diagnose CML.

CBC shows high WBC (over 100,000) | Philadelphia chromosome

What will the labs show with CML?

LAP is low | LDH, uric acid and B12 are elevated

How do you treat the chronic phase of CML?

Imatinib | If young, allogeneic stem cell transplant

How do you treat the blast phase of CML?

Imatinib Dasatinib + hematopoietic stem cell transplant Clinical trial

How do you diagnose hairy cell leukemia?

Bone marrow bx or smear shows hairy cells | TRAP stain

What is the first line tx for hairy cell leukemia?

Nucleoside analogs ->> cladribine = adenosine analog (toxic to neoplastic cells)

What are the other tx options for hairy cell leukemia?

Splenectomy | INF alpha

How do you diagnose lymphoma?

Excisional bx of a lymph node AND extra nodal site

What should be done in patients with HIV if you suspect lymphoma?

CSF exam

How do you diagnose mycoses fungoides?

Clincal Histology of skin lesion EM showing sezary cells

What is the mainstay of tx of mycoses fungoides?

Phototherapy

What can be used for more severe or extensive mycoses fungoides?

Radiotherapy

How do you diagnose Hodgkin's lymphoma?

Excisional lymph node biopsy

What are the diagnostic criteria for multiple myeloma?

Greater than 10% plasma cells in the bone marrow and/or histo proven plasma cell infiltration M Protein in the serum or urine Lytic bone lesions

How do you evaluate a patient for multiple myeloma?

Skeletal survey Bone marrow bx Serum and urine protein electrophoresis CBC

What is used in the initial tx of multiple myeloma?

Melphalan (alkylating agent) | Prednisone

How do you diagnose Waldenstrom's macroglobulinemia?

Bone marrow bx and aspirate Shows Dutcher bodies (PAS+ IgM depositis around the nucleus) Serum and urine protein electrophoresis and immunofixation

What is the tx for Waldenstrom's macroglobulinemia?

Plasmapheresis | Chemo

How do you confirm the dx of amyloidosis?

Bx with Congo red stain

What is the tx of amyloidosis?

Experimental chemo | Transplant

How do you diagnose neutropenia?

CBC with ANC

What do you do if there is associated anemia or TCP with neutropenia?

Bone marrow bx and aspirate

What is the best next step if patient with neutropenia has a fever?

Give broad spectrum antibiotics (cefepime)

What can be used to shorten the duration of neutropenia?

G-CSF

How do you diagnose eosinophilia?

H&P CBC with diff CSF analysis

What should you think of if have hematuria and eosinophilia?

Schistosomiasis

How do you treat HSP

Corticosteroids | Cytotoxic agents

What is tx for tumor lysis syndrome?

IVF Allopurinol Rasbaricase

How do you confirm HIT?

ELISA antibody test

How do you work up ITP?

Physical CBC Peripheral blood smear

How can you diagnose porphyria?

Aminolevunlimic acid and porphobilinogen in urine

When should gonadectomy be performed in androgen insensitivity?

After puberty

What is the tx for ITP in children with skin manifestations only?

Observation

What is the tx for ITP in children with bleeding?

IVIG or corticosteroids

What is the tx for ITP in adults with platelet count less than 30,000 without bleeding?

Obs

What is the tx for ITP in adults with bleeding or platelet count less than 30,000?

IVIG | Glucocorticoids

What is the test for early detection of hemophilia arthropathy?

MRI

What is the prophylaxis for hemophilia arthropathy?

Factor XIII or IX

What is the gold standard lab test for hereditary spherocytosis?

Eosin-5-maleimide binding test with the Acidified glycerol lysis test

How do you diagnose amyloidosis?

Tissue bx

What is the treatment for TTP?

Emergent Plasma exchange

What is cryoprecipitate used for?

Von willebrand disease Factor 8 deficiency Fibrinogen def

When is a platelet transfusion given?

Platelets less than 10,000 | Bleeding and platelets less than 50,000

What is the tx for primary CNS lymphoma?

Brain irradiation

What is the tx for neurocysticercosis?

Albendazole

How long is fluconazole administered for HIV patients for cryptococcal meningitis?

Until CD4 is greater than 100 for a year

How long should antiretroviral therapy be deferred for after tx if cryptococcal meningitis?

2-8 weeks