Hematology Flashcards
Hyperpigmentation of skin
Fanconi anemia, dyskeratosis congenita
cafe-au-lait macules
Fanconi anemia
Jaundice
Hemolysis
Petechiae, purpura
BM infiltration, autoimmune hemolysis, autoimmune thrombocytopenia, HUS
Frontal bossing of head
Thal major or Sickel Cell anemia
Maxillary hyperplasia
Thal Major, sickel cell anemia
Microcephaly and microphthalmia of eyes and ABSENT thumbs
Fanconi anemia
Retinopathy of eyes
Sickle Cell Dx
Glossitis
B12 Deficiency
Hyperpigmentation of mouth
Peutz-Jeghers (GI blood loss)
Telangiectasia of mouth
Osler-Weber-Rendu (GI blood loss)
Leukoplakia of mouth
Dyskeratosis congenita
Splenomegaly
SCD, thalassemia, immune hemolytic anemia, hereditary spherocytosis, leukemia, lymphoma, EBV, portal HTN
Hepatomegaly
SCD, leukemia, lymphoma
Absent radii
Thrombocytopenia-absent radius syndrome
Triphalangeal thumb, shield chest, cleft lip
Diamond Blackfann
Spoon nails
Fe deficiency: very rare
What disease will you see Heinz bodies in? What are they?
In G6PD deficiency and thalassemia; it’s aggregate of denatured hemoglobin; also see bite cells
When do we see Helmet cells?
THey are fragmented RBCs seen in traumatic hemolytic conditions: DIC, HUS or TTP
When so we see basophilic stippling?
In lead poisoning, thalassemias; from ribosomal precipitates dispersed throughout the cytoplasm.
When do we see Howell-Jolly bodies
These are seen in sickle cell and asplenic pts; its nuclear remnants or RBCs that the spleen usually removes
What lab abnormalities are seen in ITP?
What treatment can we use?
Plats less than 20,000, otherwise normal CBC with normal bone marrow aspirate.
Mild ITP is self limited while significant bleeding you can use IVIG and corticosteroids
15 mo old with recent viral illness develops pallor and goes into PCP. CBC shows HgB= 5 and retic = 0.2% with normal RBC adenosine deaminase levels. Dx and treatment
Transient erythroblastopnenia of childhood; triggered by viral infection; see profound anemia and reticulocytopenia but normal RBC ADA levels; may need 1 to 2 transfusions but this is self limited
How do you tell the difference between Transient erythroblastopenia of childhood and Blackfann
Labs in TEC show low RBC and low retic as does Blackfann BUT TEC has normal RBC ADA while Blackfann has low ADA.
Also, TEC triggered by viral illness around 1.5-3 yo in otherwise healthy child while Blackfann presents at infancy with frontal bossing, triphalangeal thumbs, shield chest and short stature
8 mo old with elevated hemoglobin H2 and mild microcytic anemia
B-Thal trait
You have a high index of suspicion that your patient has G6PD defiency, he just came in with dark urine, jaundice and pallor after being treated with TMSX for an ear infection. What test do you order to confirm your diagnosis? What would you see on peripheral blood smear?
Order G6PD enZ activity… but wait several weeks AFTER the episode as patient will have transiently elevated G6PD as newly produced RBC will have normal amt of enZ
Smear shows Heinz bodies with bite cells
What is the most common cause of retinoblastoma? Does t have a good rate? If inherited, what is the patient at increased risk for later in life?
Most common sporadic mutation and 90% of those cases will result in cure but often loss of vision in that eye. If inherited patient is at risk for soft tissues tumors later in life such as osteosarcoma or melanoma
What is fetomaternal transfusion and how do you diagnosis it?
Patient has normal retic but will appear pale; often occurs at time of delivery. Test is the Kleinhauer Betke stain for fetal hemoglobin containing RBCs in mom’s blood.
When do you see physiologic anemia of the newborn?
Seen 6–8 weeks of age: at birth erythropoeisis stops and you see progressive decline in HgB
Which conditions present with spherocytes on peripheral smear
hereditary spherocytosis, G6PD deficiency, ABO incompatibility.
*Babies with neonatal hyperbili is seen in pt’s with hereditary spherocytosis
How do you tell ABO incompatability and hereditary spherocytosis apart
Need to look at blood types of mom and baby as well as direct Coombs test on infant
What factors are tested in PT test
Is PT prolongued or normal in Hemophilia A or B?
I, II, V, VII and X
NOT prolongued; hem A (def VIII) and Hem B is (IX) thus PT is normal
What happens to PT, PTT and thrombin time in Vit K deficiency
Vit K is low II, VII, IX, X
will have prolongued PT, PTT but Thrombin time is normal as that is test for conversion of fibrinogen–>fibrin which is unaffected. Will also have normal plat count
What is vWB role in clot formation?
What meds can help patient in bleeding episode with vWB?
clot formation by adhering to areas of vascular damage adn causes plats to attach and activate.
Use DDVAP or desmopressin to transiently increase levels of vWB
How does osteosarcoma usually present? What does it look like on XRAY? What sydnromes is it associated with?
In second decade of life with localized swelling mostly in metaphyses of long bones. Sunburst on xray and associated with Li-Fraumeni, Paget or prior radiotherapy
patient comes in with high suspicion for Hodkins, what initial test would you want to do? What would you do to confirm diagnosis?
Get a radiograph of chest; mediastinal mass increases likelihood for Hodgkins; especially in addition to findings of fever, weight loss, malaise and itching
Confirm with node biopsy
What does a peripheral smear look like in child with folate deficiency
Hypersegmented PMNs
