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Flashcards in Hematology Deck (18):
1

child with the history of repeated infections, failure to thrive and anemia. His older brother also has same condition. What is the most likely diagnosis?

A. Nutritional anemia

B. Leukemia

C. Lymphoma

D. Haemoglobinopathy

Answer: D ?

Repeated infection, failure to thrive, anemia and family history of same condition make hemoglobinopathy the best answer.

2

During delivery, when the doctor cut the umbilical cord, bleeding doesn’t stop. Which of the following factors is deficient in this case?

A. Factor x

B. Factor xi

C. Factor xii

D. Factor xiii

Answer: d

The bleeding diathesis in inherited factor xiii deficiency is severe in most patients. Bleeding from the stump of the umbilical cord within the first days to weeks of life is a characteristic sign.

3

6 year old child with fever, malaise, lymphadenopathy and hepatosplenomegaly. Shown is the cbc and bone marrow aspiration slide. What is the most likely diagnosis?

A. Sickle sequestration.

B. Leukemia.

C. Malaria.

D. Leishmaniasis.

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Answer: b

The diagnosis of acute lymphoblastic leukemia (all) is made when at least 30% lymphoblasts or 20% lymphoblasts are present in the bone marrow and/or peripheral blood.

slides  should  be  stained  with myeloperoxidase (MPO) (or Sudan black) and terminal deoxynucleotidyl transferase  (TdT), unless another method  is  used,  such  as  flow  cytometry.  

Bone  marrow  samples  should  also  be  sent  for  flow  cytometry and  cytogenetics

Approximately 15% of patients with ALL have a t(9;22) translocation (i.e, philadelphia [ph] chromosome).

4

Neonate  with  physiological  jaundice  her  parent  brought  the  baby  because  the  color  changed  from  yellow  to  greenish what  you  suspect  disease:  (i’m  not  sure  about  q) A.  Abo 

B.  G6pd 

C.  Due to oxidation  of  bilirubin   

D.  The treatment  start  to  work   

Answer : c !

5

Thalassemia  causing  stillbirth,  which  type  is  it?  

A.  Two normal beta  chains  and  2  normal  alpha  chains  

B.  Two normal beta  chains  and  4  abnormal  alpha  chains  

C.  2 abnormal beta  chains  and  4  normal  alpha  chains

D. 4 abnormal beta chains and 2 normal alpha chains

 

Answer:  b

This  is  alpha  thalassemia  major  this  will  cause  loss  of  all  alpha  chain  and  it  will  lead  to  hydrop  fetalis  and  consequently  cause stillbirth. 

6

Child  presented  with  petechiae  and  his  platelets  is  15,  otherwise  healthy.  What  will  you  do  for  him?  

A.  lVIG 

B.  Splenectomy

C.  observation 

D.  Steroid 

 

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Answer  c : observation 

  in  80%  of  cases  acute , self limiting  with spontaneous recovery within  6-8 weeks

Treatment  with  IVIg  or  Prednisone  if  mucosal  or  internal  bleeding,  platelets  <10,  or  at  risk  of  significant bleeding .

Life-threatening  bleed:  additional  platelet  transfusion  ±  emergency  splenectomy Supportive RX : avoid contact sports and ASA/NSAIDs

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7

Child  eating  a  lot  of  milk  but  he  does  not  eat  meat,  mcv  hypochromic  microcytic  anemia  ,  how  will  you  manage  this  child?

A.  Oral vitamins  +  iron

B.  Trial  of  iron  then  then  observe

C.  Folic  acid

D.  Iron  

 

Answer: a 

8

16  yr  from  aferica  (ginia)  with  painless  neck  mass  for  5  weeks  developed  cough  ,  fever  ,  urs:

A.  Burkit  lymphoma  

B.  Infectious  mononucleosis  

C.  Hugging  lymphoma

D. Lym dx

 

Answer:a

  Burkitt  lymphoma,  or  small  noncleaved  cell  lymphoma,  is  a  highly  aggressive  b-cell  non-hodgkin  lymphoma  characterized  by  the translocation  and  deregulation  of  the  c-myc  gene  on  chromosome  8.   

http://emedicine.medscape.com/article/1447602-overview

9

10  years  old  girl  presented  with  fatigability,  diarrhea  and  glossitis.  What  is  the  diagnosis?  

 

Answer: vitamin B12 defeicncy 

Macrocytic  Anemia

All  anemia presents with  fatigue,  including macrocytic anemia,

·Vitamin B12 deficiency presents with neurological findings as well.

-The most common is  peripheral  neuropathy,  but  any  form  of  neurological abnormality can develop at any part of the peripheral or central nervous system.

-The least common is dementia.

-Neurological problems resolve with treatment if they have been present for a short period of time.

·B12 deficiency also causes a smooth tongue (glossitis) and diarrhea.

· Folate  deficiency:  This  does  not  present  with  neurological  problems.

 

Diagnostic Testing

· Best initial test:  CBC with peripheral blood smear  (look  for  hyper segmented neutrophils and oval cells). The average number of lobes in the normal white cell is 3.5. If the average is > 4 if more than 5 percent of the cells have > 5 lobes, the patient has  megaloblastic  anemia  as well as macrocytosis.

Oval  cells  will be visible on the peripheral smear as well.

bilirubin  level and  LDH,  B12 deficiency raises LDH and indirect bilirubin by destroying red cells early, as they come out of the bone marrow. This phenomenon is called “ineffective erythropoiesis,” and it is why the reticulocyte count is low. Although the marrow itself is hypercellular, B12 deficiency creates a molecular defect that breaks down the cells just as they leave the marrow.

The  reticulocyte count will be decreased.  

Most accurate tests:  Low B12 level  (for B12 deficiency) and  folate  level  (for folate deficiency).

Note: Up to 30 percent of those with B12 deficiency can have a  normal  B12 level, because transcobalamin is an acute phase reactant and  any  form  of  stress  can  cause  its  elevation.  If  you  suspect  B12  deficiency but the B12 level is normal, order a  methylmalonic  acid  level.  Homocysteine levels go up in both vitamin B12 deficiency and folate deficiency.

 “What is the next best test to confirm the etiology of the B12 deficiency?” antiparietal  cell  antibodies  and  anti-intrinsic  factor  antibodies. These confirm pernicious anemia as the etiology of the B12 deficiency · Schilling’s test is an older, rarely done method of confirming the etiology and is not necessary if the antibodies are present.

Treatment B12 deficiency and folate deficiency are treated with  replacement.

Note : Metformin blocks B12 absorption.

After B12 replacement therapy: Reticulocytes improve first , Neurological abnormalities  improve last

 

10

8  years  old  boy  with  petechiae  all  over  his  body.  Lab  results:  low  platelets  and  high  creatinine level.  what  is  the  diagnosis?

A.ITP

b. TTP

 

Answer: ??

HUS if  no  fever  or  altered  mental  status

HUS = Microangiopathic  hemolytic  anemia  +  thrombocytopenia  +  renal  failure  

TTP= HUS + fever and/ or altered mental status. 

11

What can  increase  fetal  hemoglobin  in  sickle  cell  anemi  (no  hydroxyurea  in    the  options) : 

5-azacytidine, butyric acid, erythropoietin and hydroxyurea 

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12

child  with  normochromic  normocytic  anemia +  splenomegaly.  Blood  smear  was  attached  showing  clear  spherocytosis.  which  of  the  following  will  be  abnormal?

A. PLT

B. Retic

C. WBC 

D. MCV 

 

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Answer: B 

Hereditary  Spherocytosis

This condition presents with:

1-Recurrent episodes of hemolysis

2-Splenomegaly

3- Bilirubin gallstones

3- Elevated  mean  corpuscular  hemoglobin  concentration  (MCHC)

Diagnostic  Testing

The most accurate test for hereditary spherocytosis is an  osmotic  fragility  test.

Treatment

Splenectomy  will  prevent  hemolysis  since  the  cells  are  destroyed  in  the  spleen.

Note : 

Hereditary spherocytosis is the genetic loss of both ankyrin and spectrin in the red cell membrane. Ankyrin and spectrin are the basis of the cytoskeleton that maintains the RBC membrane in its biconcave disc. Without this cytoskeleton, the RBC pops into a sphere. 

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13

12  yo  ,  mild  jaundice,  splenomegaly  ,  echogenic  shadow  of  gall  bladder  ?

 A. SCA

B. Thalassemia

C.hereditary  spherocytosis  

 

Answer: C. hereditary  spherocytosis   

14

Child with URTI is complaining of bleeding from nose, gum and bruising the treatment is: A-Prednisolone

B-IVIG

 

Answer : A

Explanation:  

-  Common medications  used  to  treat  idiopathic  thrombocytopenic  purpura  include:

●  Corticosteroids.The  first  line  of  therapy  for  ITP  ,  usually  prednisone,  which  can help  raise  platelet  count  by  decreasing  the  activity of  immune  system.  Once the platelet  count  is  back  to  a  safe  level,  you  can  gradually  discontinue  taking  the  drug..

●  Intravenous immune globulin (IVIG)  If there is critical  bleeding  or need  to  quickly  increase  of  blood  count  before  surgery .

●  Thrombopoietin  receptor  agonists.  The  newest  medications  approved  to  treat  ITP  are  romiplostim  (Nplate)  and  eltrombopag  (Promacta).  These  drugs  help  bone  marrow  produce  more platelets,  which  helps  prevent  bruising  and  bleeding.  Possible  side  effects  include  headache,  joint or  muscle  pain,  dizziness,  nausea  or  vomiting,  and  an  increased  risk  of  blood  clots.

 Biologic  therapy.  Rituximab  (Rituxan)  helps  reduce  the  immune  system  response.  It's  generally used  for  people  with  severe  ITP,  and  in  those  who  corticosteroids  don't  help.  Possible  side  effects include  low  blood  pressure,  fever,  sore  throat  and  rash 

15

5  years  old  girl  presenting  with  hepatospleenomegally,  pale,  decreased  level  of  conscious,  V\S indicating  shock labs:  Hb:  5  Platlets:  65

what's  your  important  next  investigation?

A-  Abdomen  US

B-  Reticulcytes  count*  (patient  has  spleenic  sequestration)

C-  BM biopsy

Answer : B

Acute  splenic  sequestration  crisis  ASSC  is  characterized  by sudden drop  in  hemoglobin  concentration,  tender  splenomegaly,  thrombocytopenia,  and  reticulocytosis  . ( suddenly  developed  tender  splenomegaly,  high  fever,  acute  anemia,  thrombocytopenia,  leukocytosis, jaundice,  hypoxia,  and  tachycardia ) 

Effective  circulating  blood  volume  is reduced,  sometimes  leading  to  hemodynamic  instability.  Fever,  leukocytosis,  splenic  rupture,  and  death may  occur.  

16

Sickler PT with recurrent cholecystitis what is management:

   

cholecystectomy

17

Case of SCA . What is the most important on long term ?

A.Penicilline

B.Hydroxyurea

 

Answer:A 

18

Long  case  about  child  2  months  with  respiratory  symptoms  his  brother  dies  from  pneumonia  and her  sister  doesn’t have  any  medical  hX.  Investigations.  ShowT  cell  0   low all IG  

 

Dx Not clearQQ