Heme (New) Flashcards
A 53 yo has Auer rods and >20% blasts in bone marrow. WBC is >100K. Dx?
Acute Myeloid Leukemia (AML)
In what type of anemia would you find pancytopenia? What is that?
- Aplastic Anemia
- All 3 blood cells low
What is the treatment for Primary Immune Thrombocytopenia in a patient with <30K platelets and is actively bleeding, has RFs, or does not respond to steroids (or was CI)?
IVIG
What labs are found in von Willebrand Disease (VWD)?
- ↓ vWF
- ↓ factor VIII (causes abnormal platelet function BUT normal platelet count)
- prolonged BT
- prolonged PTT (in 50%)
- normal PT
What labs are found in Hemophilia A and B?
- ↑ PTT
- normal PT and platelets
- ↓ Factor VIII or IX
What is the treatment for von Willebrand Disease (VWD)?
Desmopressin
What is the treatment for Hemophilia A and B?
Factor VIII or IX, cryoprecipitate
What is the appropriate treatment for a 52 yo man with severe aplastic anemia?
Anti-thymocyte globulin + Cyclosporine, and Prednisone
What is the treatment for Primary Immune Thrombocytopenia in a patient with <30K platelets and no bleed?
Steroids
How is anemia of chronic disease diagnosed?
Normal or ↑ Ferritin* + ↓TIBC*, ↓ Fe
A bone marrow biopsy shows hypocellular + fatty infiltration. What is the likely diagnosis?
Aplastic Anemia
Microcytic, hypochromic RBCs with ↓ Fe, ↑ total iron binding capacity (TIBC), ↓ ferritin levels would make you think:
Iron Deficiency Anemia
What are the lab findings for a B12 (Cobalamin) Deficiency?
- ↑ homocysteine
- ↑ methylmalonic acid (MMA)
- hypersegmented neutrophils
With recurrent DVTs and pulmonary embolism, esp. young, one would think:
Factor V Leiden
What is the earliest finding in iron deficiency anemia?
↓ serum ferritin (low stores)
↑ MCV and megaloblasts. Dx?
Folate Deficiency
Suspect _____ if renal failure in kids with a prodrome of diarrhea associated with thrombocytopenia on the CBC
Hemolytic Uremic Syndrome (HUS)
In repeated spontaneous abortions, recurrent DVTs or CV events, one would think:
Antiphospholipid Antibody Syndrome
How is Factor V Leiden diagnosed and confirmed?
Activated Protein C assay. If (+) → confirm with DNA testing.
What is the only disorder that will cause an ↑ in mean corpuscular hemoglobin concentration (MCHC)?
Hereditary Spherocytosis
ART (Autoimmune hemolysis, Renal failure, Thrombocytopenia) + Fever + Neuro abnormalities = (FAT RN). Dx?
Thrombotic Thrombocytopenic Purpura (TTP)
↓ fibrinogen, ↑ PT/PTT/INR, severe thrombocytopenia; ↑ fibrinolysis (D-dimer). Dx?
Disseminated Intravascular Coagulation (DIC)
A 5yo male presents with purpura, arthritis, abdominal pain and hematuria. Mom reports a URI a couple weeks ago that has since cleared. What is the likely diagnosis?
Henoch-Schonlein Purpura
How is Henoch-Schonlein Purpura diagnosed?
Definitive: renal biopsy showing mesangial IgA deposits in granular pattern
How is Henoch-Schonlein Purpura treated?
ACE-I and early steroid therapy reduced proteinuria
Alpha Thalassemia is seen mostly in what ethnicities?
Asian > African > Mediterranean
Beta Thalassemia is seen mostly in what ethnicities?
Italian or Greek descent
How is Beta Thalassemia (minor) diagnosed?
Microcytic hypochromic cells, ↑ Hgb A2, target cells
How is Beta Thalassemia (MAJOR) diagnosed?
Severe anemia (Hgb ~6) ↓ MCV ↑ HgbF Codocytes (target cells) Hepatosplenomegaly
X-ray showing hyperdense lines at metaphyses, also know as “___ lines” are due to:
- “Lead”
- Lead Poisoning
What is the treatment for lead poisoning?
Chelation therapy → PO Succimer or IV CaNa2EDTA (Calcium disodium edetate, given after Dimercaprol)
How is Hereditary Spherocytosis diagnosed?
Osmotic fragility test
The body stores enough folic acid to last:
4-5 months
On a peripheral smear, what would be seen in Hereditary Spherocytosis?
Spherocytes and Howell-Jolly body
How is Hereditary Spherocytosis treated? What about in newborns with jaundice?
- Daily folic acid
- Phototherapy or exchange transfusion
Labs: ↑ RBC mass, ↑ Hct, ↑ Hgb, abnormal proliferation of all 3 myeloid cell lines; Leukocytosis and thrombocytosis. Dx?
Polycythemia Vera
Multiple, painless, peripheral lymphadenopathy; noncontiguous spread + GI Sxs in an immunocompromised pt is seen in:
Non-Hodgkin’s
HIGH platelets, splenomegaly, pseudohyperkalemia, ↑ bleed time and Erythromelagia (burning and erythema of extremities) is seen in:
Thrombocythemia (Thrombocytosis)
A 50 yo with splenomegaly and Philadelphia chromosome. Dx?
Chronic Myelogenous Leukemia (CML)
↑ fasting Transferrin and Ferritin; ↓ TIBC. Dx?
Hemochromatosis
Treatment for Hemochromatosis
- Phlebotomy
- Deferoxamine chelation
A 55yo male presents with RUQ pain, weakness, joint pains, sclera icteric and bronze extremities. What tumor marker should be checked in this patient?
Dx: Hemochromatosis
(B symptoms) Fevers, night sweats, weight loss > 1 mo and painless, localized, single group of lymph nodes are found in:
Hodgkin’s
Elderly pt. with bone + back pain refractory to treatment, labs showing monoclonal antibody spike and x-ray showing lytic lesions. Dx?
Multiple Myeloma
What is the mutation for Polycythemia Vera?
Janus kinase 2 gene (JAK2)
Child with lymphadenopathy, hepatosplenomegaly and >20% Blasts. Dx?
Acute Lymphocytic Leukemia (ALL)
What d/o has increased systemic absorption of iron, resulting in iron saturation and fibrosis in various organs?
Hemochromatosis
What is the treatment for Thrombocythemia (Thrombocytosis)?
- Antiplatelet (Anagredlide and low-dose ASA)
- Hydroxyurea (severe)
What is the treatment for Chronic Myelogenous Leukemia (CML)?
- Curative: Allogeneic hematopoietic stem cell transplantation (HSCT)
- Imatinib
A 50 yo with isolated lymphocytosis (>20 K) and smudge cells. Dx?
Chronic Lymphocytic Leukemia (CLL)
A 67 yo presents with SOB, fatigue and easy bruising. Labs show ↓ RBCs, WBCs and platelets, ↑ MCV, ↑ red cell width (RCW), ↓ retic count and ↑ blasts. What is the likely diagnosis?
Myelodysplastic Syndrome
What diagnosis present with splenomegaly?
Autoimmune Hemolytic Anemia Polycythemia Vera Thrombocythemia (Thrombocytosis) Acute Myeloid Leukemia (AML) Chronic Myelogenous Leukemia (CML)
What diagnosis present with hepatosplenomegaly?
Anemia Acute Lymphocytic Leukemia (ALL) Beta Thalassemia Major (Cooley’s anemia) Cirrhosis Toxoplasmosis Cytomegalovirus Histoplasmosis (immunodeficient)
What is prophy for sickle cell disease in kids and when is it started?
- Penicillin V (start @ 2 mo until 5yo)
- Folic acid (start @ 1 yo)
A patient has a platelet count of 10,000, prolonged prothrombin level, and the presence of fibrin split products. Dx?
Disseminated Intravascular Coagulation (DIC)
Which diagnoses have a (+) Coombs test?
- Autoimmune Hemolytic Anemia
- Primary Immune Thrombocytopenia (formerly ITP)
What 2 conditions present with fragmented RBCs on peripheral smear?
Disseminated intravascular coagulation (DIC) and Thrombotic thrombocytopenic purpura (TTP)
What d/o is caused by defective synthesis of protoporphyrins and causes iron to accumulate in mitochondria?
Sideroblastic Anemia
What is the treatment for Pernicious Anemia?
IM Cobalamin (B12) Q month
With an elevated prothrombin time (PT) but a normal partial prothrombin time (PTT), what is the most appropriate pharmacotherapy?
Vitamin K
What is a know RF for Myelodysplastic Syndrome?
Previous radiation
What are Prussian blue stains use for?
Evaluate iron stores and check for Sideroblastic Anemias
In what condition is Rouleaux formation is seen?
Multiple Myeloma (Plasmacytoma)
In Sickle Cell Disease, what labs and smear are seen to diagnose?
↑ reticulocytes
↓ Hct, Hgb
Smear: Howell-Jolly body
What disorders need cryoprecipitate if they are bleeding?
- von Willebrand disease
- Hemophilia A
- Disseminated intravascular coagulation (DIC)
What disorder is shown to have isolated lymphocytosis in asymptomatic patient?
Chronic Lymphocytic Leukemia (CLL)
What are some causes of a prolonged Partial Prothrombin Time (PTT)?
- vWD
- Hemophilias
- Disseminated Intravascular Coagulation (DIC)
- Newborn Hemorrhagic Disease / Vitamin K Deficiency
- Heparin use
What are some causes of a prolonged Prothrombin Time (PT)?
- Disseminated Intravascular Coagulation (DIC)
- Newborn Hemorrhagic Disease / Vitamin K Deficiency
- Warfarin use
What electrolyte abnormality is most commonly seen in a patient with multiple myeloma?
Hypercalcemia
What is the treatment for Tumor Lysis Syndrome?
Allopurinol and IVF
