Question

HEMATOLOGY

CASE 1: A 20-year old patient who was previously diagnosed with anemia and being treated with iron tablets for the last 10 days. On her second visit after iron tablet treatment, the patient felt much better physically and her blood smear showed normal WBC differential and reticulocyte count higher than first visit. Answer the following questions regarding this patient after the iron tablet treatment.

6. She is likely suffering from iron deficiency anemia and responding to the iron therapy. (a) True; (b) False

7. Her bone marrow C: F ratio should be: (a) higher; (b) lower than normal ​​​​​​​

CASE 2: History of Present Illness: A boy 3 years of age was admitted with a liver abscess (pus). He had been ill with many infections since the age of 1 month. Types of infections included recurrent pyoderma (skin infection), several episodes of pneumonia, perirectal (around the rectal area) abscess, and mastoiditis (ear infection). Organisms that were recovered from these sites of infection from several occasions were either Staphylococcus or Pseudomonas, with one time being Klebsiella (gram negative bacteria). Despite these episodes of infection, the patient experienced normal growth and development. Immunizations had been given without incident; in particular, he had no difficulties with administration of live virus vaccines. The family medical history showed an older brother and a cousin (son of a maternal aunt) had suffered similar genetic disease with chronic infections, and one had died of chronic, gram-negative pneumonia.

Physical Examination: The boy appeared to be generally well. His skin was covered with scattered areas of crusted scabs. Gram stain of the moist base of one such scab revealed gram-positive organisms (Staphylococcus). Lymph nodes in all areas were enlarged to 3 to 6 cm, and those in the neck were firm and tender. The tympanic membranes (inside the ear) were scarred, and the right one was perforated (hole). The lungs on the day of examination were normal. The liver was enlarged to 8 cm below the lower rib margin, and the spleen was so large that it extended into the pelvis. The patient was not taking any medication or drug for the last 6 months and did not show any symptoms of being anemic.

Laboratory Data: The hemoglobin concentration was 18 g/dl, and the erythrocyte morphology was fairly normal. The total white blood cell count was 21,000/µl, with 11,000/µl neutrophils. The differential of WBC did not reveal monoclonality, meaning cell of similar one type of morphological appearance.   The neutrophils contained toxic granules, Döhle bodies (abnormal bodies inside the cytoplasm), and vacuoles (All indicate signs of bacterial infection). The platelet count was 340,000/µl.

8. Question: How likely this patient suffer genetic disease inherited from the parents?

a)   Likely

b)   Not likely

Answer 1

ANSWER 6- TRUE

Patient is likely suffering from iron deficiency anemia , as her reticulocyte count and differential count increased comparatively to her first visit, after taking iron tablets.

A low reticulocyte count occurs when body does not have enough iron stored to make red blood cells.

ANSWER 7- Ratio is higher than normal.

ANSWER 8- Patient is likely suffering from a genetic disease , inherited from parents as older brother and son of maternal aunt also did suffer from same disease.

• As the case history suggest this could be MyD88 deficiency, which is an inherited dissorder of immune system, affecting body's innate immune response.This deficiency leads to abnormally severe,frequent infections due to pyogenic bacteria , which causes pus. Most common organisms involved are Streptococcus pneumoniae, Staphylococcus aureus and Pseudomonas aeruginosa.