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Internal Medicine > Heme Onc > Flashcards

Heme Onc Flashcards

1
Q

What enzyme is deficient in cutanea tarda?

A

Cutanea Tarda Prophyrea (Defect in Heme production) Photodermatitis + Pink/Brown Urine. They lack Urophpohyrinogen Decarboxylase. Elevated Uroporphyria III can be present in Blood, Urine or Stool.

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2
Q

Most common cause of Sideroblastic Anemia? What will the iron studies show? Most accurate test? Rx?

A

Acquired or Hereditary. Alcohol is Most Common. Other causes: Aminolevulunic Acid Synthase Defect (heredity) defect can be aquired with lead poisoning.) Cholorophemical, INH. Iron Studies: low MCV, low serum iron, high ferritin, low TIBC. Most accurate: Prussian Blue Stain (Ringed sideroblast will show up in the bone marrow.) Remove offending agent. Can give B6 in some cases.

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3
Q

The Rx of Beta Thalassemia major ? What else do you need to add?

A

Transfusion + Chelation with PO Deferasirox Or IM Deferoxamine.

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4
Q

What test Confirms Pernicious Anemia? What test Confirms B12 deficiency?

A

Anti-Intrinsic factor and Anti-parietal cell antibodies. elelvated MAMA level + homocystein level, B12 level confirms but takes too long to return.

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5
Q

Common complication of B12 and Folate Replacement?

A

Hypokalemia, Hypomagmesium due to cell building.

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6
Q

Pt presents with Pancytopenia and Dark urine. Anemia is WOSRE at NIGHT. They can wake up with HA. Most accurate test? Rx? Complication?

A

Paroxysmal Nocturnal Hemoglobinuria (PNH) (Caused by a cell membrane defect in PIG-A ->increased complement binding -> intravascular hemolysis especially at night when 02 sats drop-> they lyse in an acidic enivornment.) Most accurate test: CD55 or CD59 levels that would be Low Rx: 1. Prednisone and Eculizumab (inactivates C5) Complications : 1. Thrombosis (Budd Chiari syndrome) - from destruction of the platelets leaving platelet fragments causing activating the coagulation cascade (Most common cause of death.) Rx with anticoaugs.

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7
Q

Name 2 dz associated with IgM mediated hemolysis? Test? Rx? Why will prednisone and splenectomy not help this disease?

A
  1. Mycoplasma pneumonia. 2. EBV Test: Direct (check for IgM bound RBCs) or Indirect Coombs (check for Antibodies in Serum) Rx : Rituxmbab + Stay warm (also used for NONhodgkin and Rheumatoid.) Prednisone and Splenectomy will not Rx this disease because the hemolysis is by the liver (kupfer cells.)
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8
Q

Most accurate Test for Autoimmune Aplastic Anemia? (Other causes: Drugs: PTU, Methamizole, Carbamezapine, Benzene, Radiation, Toxins, HIV, EBV, CMV, Paro 19) Rx for Young people? Treatment for old people?

A

Autoimmune is caused by the T cells attacking the bone marrow) Rule out causes of pancytopenia. Test: Bone marrow biopsy will show many fat cells and hypocellular bone marrow. Rx : 1. Eliminate Causative Agent. 2. Allogenic Bone Marrow Transplant. 3. If too old age then Anti-thymocyte (ATG) globulin and Cyclosporine (lymphona, infection).

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9
Q
  1. What is the initial test of Sickle cells Dz ? What is the most accurate test? Acute Rx? Chronic Rx? Common 2. Complication of patients with sickle cell dz? Best test for this?
A

SS Valproate to glutamate. Best initial test Peripheral smear. Most accurate test Electrophoresis. Oxygen, hydration Opioids for pain crisis, transfusion if needed. Chronic: Folic acid, Pneumovax, Hydroxyurea (increases hemoglobin F) - use if>3 pain crisises in a year.
2. Papillary necrosis. Do CT scan to show bumpy contour of the papillae. Acute chest syndrome, Osteo (salmenella), Aplastic Crisis, Hyperhemolytic crisis, splenic sequestration.

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10
Q
  1. Acute Myelogenous luekemia (AML) and Acute Lymphocytic Leukemia (ALL- most common childhood malignancy) What are the best initial test ? What is the most accurate test for these? 2. Rx for AML? 3. Rx for ALL? 4. What is the risk associated with this disease and what is the treatment? 5. Prognosis is based on what?
A
  1. Best initial Test is CBC and Blood Smear. Most accurate Test: Bone Biopsy/Aspiration/Flowcytometry. Auer Rods present in AML Type M3-APL and is associated with DIC. ALL is TdT positive. 2. Rx: Chemo Until Remission and then induction (85% of kids achieve remission). 3. Rx: ALL needs Intrathecal Methotrexate, ATRA for AML Type M3 (APL). 4. Luekapharesis for luekostasis. 5. Prognosis based on Cytogenetics. (Poor do BMT. Good then do more chemo.)
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11
Q
  1. What is the difference btw CML and Leukomoid Reaction?
A
  1. Alk phos elevated in infection (released from cells to fight infection) and decreased in CML..
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12
Q

B cells with Filamentous Projections seen on blood smear. Dx? Test Rx?

A

Hairy Cell Leukemia. Cytoplasmic projections and TRAP. Cladribine.

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13
Q

Reed-Sternberg cells. Dx? Most accurate Test? Rx? What has the worse prognosis and which has the best prognosis ?

A

Hodgkin’s disease. Best test: Excisional Biopsy (Need a full view of the whole lymph node.) Usually stage 1 and 2 can take Radiation and Chemo. Stage 3 and 4 need ABVD + MOPP therapy (exclusively with chemo) . Lymphocyte present has best and lymphocyte depleted has the worse. Relapse after chemo: Bone marrow transplant.

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14
Q

Presents with NO Reed-Sternberg cells. Divide into Stage 1 (1 node) Stage 2 ( >1 node one side of diaphragm) Stage 3 (Both sides) Stage 4 (Widespread) Dx? Most accurate Test? Rx? What is the worse prognosis?

A

Non-Hodkins Best Test: Excisional biopsy of lymphoma. Rx: CHOP (Chemo) + Rituxumab if Antibody to CD20. Burkitt has Worst Prognosis.

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14
Q

Name 3 Test for vWF?

A
  1. Increased Bleeding time (normal PT and pTT) 2. Vwf level 3. Ristocetin test.
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16
Q

Best initial test if you suspect Antiphospholipid Syndrome? Next test after that?

A

Do mixing study - it won’t correct the thrombosis because of the presence of the lupus anticoagulant. Then do Russell Viper Venom Test to confirm Antiphospholipid

17
Q

Fever, flank pain, hemoglobinuria, renal failure and DIC within 1 hr of transfusion. There is a Positive direct Coombs test. Pink plasma (Plasma free Hemoglobin). Prevention?

A

ABO incompatibility reaction (Febrile Hemolytic Reaction). Usually the type and cross match was wrong!!!! Fluids and Supportive Rx.

18
Q

Fever chills within 1-6 hrs of transfusion dx? Negative Coombs Test. Cause? Prevention?

A

Febrile NON-Hemolytic reaction. Caused by cytokine accumulation during blood storage. Prevention: Need leukoreduced blood products./ Washed Products.

19
Q

Urticaria, flushing, angioedema and pruritus within 2-3 hrs of transfusion. Dx? Cause? Rx?

A

Caused by recipient IgE antibodies and Mast cell Activation. Give EPI.

20
Q

Rapid onset of shock, angioedema/urticaria and respiratory distress within in a few seconds to minutes of transfusion. Dx ? Cause? Rx? Prevention?

A

Analphylactic caused by recipient Anti-IgA antibodies. Pts who are IgA deficient are at risk for developing anaphylaxis. Give EPI.

21
Q

Positive family hx, splenomegaly, cholecycstitis due to pigmented gallstones. MCHC > 30 Dx? Best initial Test? Most accurate Test? Rx?

A

Hereditary spherocytosis. 1. Smear (Spherocytes) Coombs negative (Remember Autoimune also presents with spherocytes) 2. Osmotic fragility test. Rx: Folic acid replacement and splenectomy.

22
Q

Mild fever and hemolytic anemia within 2-10 days after transfusion. There is a positve direct Coombs test, positive new antibody screen. Dx?

A

Delayed hemolytic reaction. Caused by anmnestic antibody response to a red blood cell antigen that the patient was previously sensitized to.

23
Q

Old man has elevated serum protein with normal albumin. He has no symptoms. No bone pain. H and H is normal. UA and urine electrophoresis is normal Dx? Next step in management?

A

Possible MGUS. Need to do metastic skeletal bone biopsy to r/o MM.

24
Q

Myeloproliferative disorders: Name Rx for each. 1. ruddy appearance, Increase hematocrit >50%, . 2. painful red hands, plt count >600,000 + jak 2 3. 3. Impaired bone marrow function, fever, hepatosplenmegaly, pancytopenia, tear drop cells, dry tap aspirate of bone Dx? Rx?

A

1.Polycythemia Vera. Serial phelbotomy, daily ASA. Hydroxyurea if severe. 2. ET Essential Thrombocytosis Hydroxyurea, Asa, Anagralide if chronic. 3. Rx supportively w/ transfusions, hydroxyurea or splenectomy if needed.

24
Q

Pre-leukemic disorder in older pts that occurs with 5q deletetion. They have Pancytopenia and Hypercellular bone marrow. Dx? Test? Rx?

A

Myelodysplastic Syndrome. Pelger-Huet Cells (Blast cells) and Anemia and Blast Cells. Hypercellular bone marrow. Rx: Transfusion and EPO.

24
Q

Test for CLL/SLL? Rx?

A
  1. Smudge cells on peripheral smear. Fludarabine + Rituximab.
26
Q

Name the Side effects: 1. Doxirubicin/Anthracycline toxcity 2. Vincristine, 3. Bleomycin 4. Cyclophosphamide 5.Cisplatin

A
  1. Dilated Cardiomyopathy 2. Neuropathy 3. Lung Fibrosis 4. Cystitis 5. Renal and Otoxicity.
27
Q

Name the transplant rejection: Occurs after days to months. Evidence tissue damage (LDH, GGT, BUN/CR). Dx? Rx?

A

Acute Rejection. Steroids + Immune modulators.

28
Q

Overproduction of IgM from malignant B cells leading to hyperviscosity. Leads to engorged blood vessels in the eye and mucosal bleeding. Pts can present with raynouds phenomenon, peripheral neuropathy and blurry vision. Dx? Most accurate test?

A

Waldenstrom Macroglobulinemia. Remember IgM is associated with cryoglobulinemia and cold agglulutins so they can have manifestations of these diseases as well. Bone marrow aspirate and biopsy which shows PAS+IgM (Dutcher Bodies.)

29
Q

Bone pain, Renal failure, Anemia. Dx? Test? Single Most accurate test? Rx?

A

Multiple Myeloma. (CRAB) Iron studies will show anemia. Smear will show Rouleaux due to the IgG paraproteins sticking together. Bence Jones Protein present on electrophoresis NOT ON DIPSTICK!!! This can lead to Renal Failure. Xray showing punched out lesions/ Skeletal survey. SPEP increased IgG and IgA give M Spike. Bone Biopsy is the Most Accurate test. Rx: Lenalidomide.

31
Q

Fever, neurological complaints, rising BUN/Cr. Increase bleeding time all other coagulation studies are normal. Dx? Rx? What not to give?

A

Thrombotic Thrombocytopenic Purpura (Defect in ADAMTs 13) FAT RN (Fever, Anemia, Thrombocytopenia, Renal failure, Neurological Complaints.) Plasmapheresis/ plasma exchange. No transfusion.

32
Q

Pt experiences abrupt onset of hemorrhage. Had viral infection 1 week ago. Dx? Test? Rx?

A

Acute Idiopathic/Immune Thrombocytopenic Purpura (IgG antibodies to platelets.) Etiology: post Viral, neoplasm, autoimmune disorders (SLE.) Test: Increased bleeding (BT) time. Decreased platelets. Rx: >30,000 observe. < 30,000 or bleeding 1. steroids 2. IVIG 3. Rituxan 4. Splenectomy

33
Q

Causes of microcytic anemia?

A

TICS (Thalassemia, Iron, Chronic dz, Sideroblastic Anemia.)

33
Q

Test for HIT?

A
  1. Serotonin Release Assay 2. Heparin induced platelet aggregation Assay 3. Heparin Antibody platelet factor 4 ELISA
34
Q

Reversal for life-threatening bleeding on warfarin ?

A
  1. Vit K + prothrombin concentrate complex 2. FFP is 2nd line.
35
Q

Name the complication of sickle cell 1. Acute, Severe Anemia + low reticulocyte count 2. Acute, Severe Anemia + high reticulocyte count 3. Anemia, Splenomegaly + high reticulocyte count

A
  1. Aplastic crisis (parovirus B19) 2.Hyperhemolytic crisis 3. Splenic sequestration

Internal Medicine (13 decks)

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