All the 'penia's
30 mo male, 2 day h/o fever, decreased activity, poor appetite. Past 6 mos has had several similar episodes. 1x hospitalized for dehydration and bacterial pneumonia. CBC shows ANC of 180. Blood culture positive for Clostridium.
What is most likely to be on exam?
A. Aphthous ulcers and stomatitis
B. Bloody diarrhea
C. Migratory arthritis
D. Bullous impetigo
E. Purulent conjunctivitis
Aphthous ulcers and stomatitis
Diagnosis: cyclic neutropenia--> high risk for sepsis from C. septicum!
15 mo old girl in custody of DCFS. Taken from home due to medical neglect. Her diet is mostly cow's milk.
Which of the following is most likely to be identified during evaluation?
A. Small RBCs w/ marked central pallor and small ring of Hgb on peripheral blood smear
B. Enlarged RBcs on peripheral blood smear
C. Small, well rounded RBCs w/o area of central pallor
D. Multiple, hypersegmented PMNs
E. Prominent basophilic stippling of small RBCs w/ central pallor
Small RBCs with marked central pallor and a small ring of Hgb on peripheral blood smear
5 mo old boy w/ macrocytosis has marked deficiency of RBC precursors in an otherwise normally cellular bone marrow. No lab evidence of Parvo B19 infection.
Which of the following is the most likely diagnosis?
A. Sideroblastic anemia
B. Transient erythroblastopenia of childhood
C. DIamond Blackfan Anemia
D. Fanconi anemia
E. Schwachman Diamond Syndrome
A. DIamond Blackfan anemia
18 mo old male from Kenya. Comes to ER w/ fever, irritability and swollen feet.
Which of the following is most likely?
A. peripheral smear w/ very high number of WBCs and Dohle bodies
B. Smear with sickled cells and polychromasia
C. Hgb electrophoresis w/ 60% HgbA and 40% Hgb S
D. smear with hypochromic, microcytic RBCs, target cells and polychromasia
B. Smear with sickled cells and polychromasia
3 yo Greek boy comes to ED due to pallor. He is markedly pale w/ HR 155bpm. Sclerae are icteric. Hgb 5.8 and dark urine.
What medicine likely caused this?
A. Montelukast
B. Cefdinir
C. PHenytoin
D. Primaquine
E. Mupirocin
D. Primaquine
Diagnosis: G6PD deficiency
A. large platelets
B. Microthrombocytes
C. Large platelets and white blood cell inclusions
D. Shistocytes
E. Toxic granulation
A. large platelets aka megathrombocytes
Patient with hgb 9.6 and peripheral smear with basophilic stippling
This findings is most often associated with?
A. Methehmoglobinemia
B. Fanconi Anemia
C. Lead Poisoning
D. G6PD deficiency
E. Diamond Blackfan syndrome
C. Lead poisoning
A 2 week old boy here for 1st eval after d/c from newborn nursery. No complications. BUT he has b/l triphalangeal thumbs, wide set eyes and a "snub nose". He is pale. Hgb 7.9.
Which lab finding is most likely?
A. Reticuloctopenia
B. Normochromic microcytic RBCs
C. Thrombocytopenia
D. Hypersegmentation of neutrophils
E. Decreased serum iron levels
A. Reticulocytopenia
Dx: Diamond Blackfan Anemia aka congenital hypoplastic anemia
-Macrocytic anemia
-have short staturs
-some w/ renal/cardiac anomoalies
Which of the following supports the diagnosis of B-thal trait when distinguishing between iron deficiency and B-thal trait?
A. elevated HgA2 and F on hgb electrophoresis
B. Microcytic RBcs
C. Hypochromic RBCs
D. Diet consisting of primarily cow's milk
E. Diet consisting of primarily goat's milk
A. elevated HgBA2 and F on electrophoresis
In B thal trait, electrophoresis shows elevated HgbA1(>3.5-8%). HgbF may be elevated as well
13 yo boy c/o fatigue after URI. He has pallor, scleral icterus and palpable spleen.
Hgb 6.7, retic 10%, unconj bili 2.8, LDH 4782.
What lab test will be most helpful at this time?
A. G6PD assay
B. Direct coombs
C. Osmotic fragility
D. EPO level
E. Hgb electrophoresis
B. Direct coombs
7 y/o w/ history of frequent nosebleeds and recurrent infections has quantitative decrease in erythrocytes, leukocytes and platelets. Lab testing shows excessive chromosomal breakage following exposure of cells to agents known to cause damage to DNA.
Which is most likely to be identified during further eval?
A. Enlarged testes
B. Hepatomegaly
C. Hyperextensible joints
D. Retinal coloboma
E. Cutaneous hyperpigmentation
E. Cutaneous hyperpigmentation
Dx: Fanconi Anemia-->bone marrow failure syndrome. Also w/ abrnomal thumbs, radial bones and skin pigmentation. Have increased risk of AML!
2 mo old girl presents for recheck of large hemangioma overlying her left cheek, extending across, above and below her left year. Has increased in size over past 3 weeks.
She is at risk for which of the following?
A. Thrombocytopenia
B. Dec C3,C4 and CH50
C. Elevated transaminases
D. Neutropenia
E. Elevated serum uric acid
A. Thrombocytopenia
Dx: Kasabach Merritt phenomenon aka hemangioma thrombocytopenia syndrome
4 mo old female w/ poor feeding and poor weight gain. Sluggish feeder. She has marked pallor and hyperdynamic cardiac exam. Hgb 2.6. Hematologist suggests Diamond Blackfan anemia.
What else would support this diagnosis?
A. Dark urine
B. Significant thrombocytopenia
C. Reticulocytosis
D. Macrocytosis
E. Marked Hepatosplenomegaly
D. Macrocytosis
Which of the following situations in a patient with SCD is least likely to require RBC transfusion?
A. ACS
B. TIA
C. Hgb < 7
D. In prep for surgery
E. Acute splenic sequestration
C. Hgb < 7
3 y/o appears lethargic and tired. BP 75/50, HR 140. Icteric sclerae are present and spleen is enlarged. Hgb is 8.2, indirect bili is 6.8. Abdominal US is positive for several gallstones and moderate enlargement of the spleen.
Which of the following additional lab results is most likely to be present upon further eval of the patient?
A. spherocytes on peripheral smear
B. Elevated ammonia
C. elevated lead level
D. positive indirect coombs
E. elevated amylase and lipase
A. spherocytes on peripheral smear
Microthrombocytopenia is seen in which of the following disorders?
A. ITP
B. Bernard Soulier
C. Wiskott Aldrich
D. May Hegglin Anomaly
E. Gray platelet syndrome
C. Wiskott aldrich
4 mo old girl pale on exam. NO rashes or bruising. Hgb 8.9. She is exclusively breastfed. Mom is healthy and vegan.
Which of the following will appear on peripheral smear?
A. Microcytosis
B. Macrocytosis
C. Howell Jolly bodies
D. Atypical lymphocytes
E. Helmet cells
B. Macrocytosis
B12 deficiency
35 wga male in NICU. Has marked shortening of both upper extremities. Plain XR shows bilateral absence of the radii w/ presence of the complete thumbs and hypoplasia of the distal humeri and shoulder girdles.
Which of the following will likely be present?
A. Stromal marrow cells w/ very few hematopoietic cells in bone marrow
B. Fragmented RBCs and burr cells
C. Macrocytic RBCs w/ hypersegmentation of neutrophils
D. Markedly reduced RBC precursors
E. Absence of megakaryoctes in the bone marrow
E. Absence of megakaryocytes in bone marrow
Dx: Thrombocytopneia- absent radius (TAR) syndrome
-Autosomal recessive, b/l absences of radii +/- ulna too
-NORMAL THUMBS!
Your 16 yo male patient w/ HgbSS is taking hydroxyurea but is having more VOCs.
Which lab finding is an expected result of hydroxyurea therapy?
A. increased in platelet count from 80k to 150k
B. Decreased in HbF from 16% to 3%
C. Increase in WBC from 9.4 to 16.8
D. A decrease in ANC from 8,000 to 1870
E. An increased in Hbs from 80% to 90%
D. A decrease in ANC from 8,000 to 1870
1 week old male presents to ER w/continuous oozing from his circ. Hgb is 7.9.
Which of the following test will confirm your diagnosis?
A. von willebrand antigen and activity levels
B. Antiplatelet antibody test
C. Disseminated intravascular coagulation panel
D. PT/aPTT/INR
E. Osmotic Fragility Testing
D. PT/aPTT/INR
Dx: Hemophilia
What is the pathophys behind this?
A. Infiltration of the bone marrow by leukemic blasts
B. Bone marrow failure w/ inadequate production of myeloid precursor cells
C. Autoantibodies to granulocytes
D. Defects in granulocyte adhesions
E. Vitamin B12 deficiency
C. Autoantibodies to granulocytes
Patient with tetralogy of fallot and bilateral radial aplasia.
Which of the following is most likely to be identified upon further evaluation of this patient?
A. Bilateral cataracts
B. Marked decrease in megakaryocytes on bone marrow
C. Hepatosplenomegaly
D. Extensive blistering of the fingers, hands and feet
E. Hypocalcemia
B. Marked decrease in megakaryocytes on bone marrow
Dx: Thrombocytopenia absent radius syndrome!
4 yo w/ h/o horseshoe kidney has hyperpigmentation on trunk and neck, several cafe-au-lait spots. Weight is 20th percentile and height is <3rd%.
Which of the following findings would suggest that she has Fanconi Anemia?
A. widening of the diploic spaces on MRI
B. A hypocellular bone marrow with with fatty infiltration
C. Evidence of fat malabsorption secondary to pancreatic insufficiency
D. A normally cellular bone marrow w/ the exception of absent red blood cell precursors
B. A hypocellular bone marrow with fatty infiltration
4 y/o develop life threatening bleeding after tonsillectomy. CBC shows platelet count of 165,000 and elevated mean platelet volume. PT is 11.4, aPTT is 35.5. Fibrinogen is 640.
What is the pathophys of this event?
A. Antiplatelet antibodies
B. Lupus anticoagulant
C. Congenital Factor 7 deficiency
D. Deficiency of glycoprotein 1b
Deficiency of glycoprotein 1b
Bernard Soulier syndrome- abnormally large platelets
16 y/o w diagnosed thrombotic thrombocytopenic purpura.
Which of the following is true about her condition?
A. The disorder is tx w/ tranfusion of RBCs
B. Condition is life threatening and must be tx w/ plasmapharesis and steroids
C. The tx is transfusion of platelets
D. Underlying cause is autoantibodies against platelets
E. RBCs on peripheral smear will be normal
B. Condition is life threatening and must be tx w/ plasmapharesis and steroids