CASE PRESENTATION
A 31-year-old Pakistani female presented with generalized body pain and low back pain. Her past medical history was remarkable for transfusion-dependent beta thalassemia (TDBT) major since childhood complicated with iron overload as per her cardia and hepatic MRI reports, receiving iron chelation therapy (Deferasirox 1080 mg daily), type II diabetes mellitus on Insulin therapy and migraine. her past surgical history includes splenectomy on 2012. She has a long history of body pain. She was recently admitted due to COVID-19 infection and discharged from quarantine facility one week earlier after a smooth course of infection.
She presented to the emergency department complaining of body pain for the last few days and dysuria. She has similar complains on and off for many years, yet the pain increased recently, and it is mainly in the hip and lower abdomen area (suprapubic), the pain increases with urination, she has also back pain for the last three months more severe at night, responds partially to analgesia, radiates to the hip and is limiting her movement. On admission vitals were:
BP 107/67 mmHg,
HR 95/min
Temperature 36.7o
Saturation 96% on room air.
Primary investigations included: Labs, MRI pelvis and hips, Abdomen US showed the following:
Labs: On admission:
Her labs revealed hypothyroidism, so she was started on Levothyroxine therapy.
X ray pelvis and left hip:
Showed decreased bone density, bilateral coxa profunda.
US abdomen showed mildly enlarged fatty liver (18.2 cm).
US pelvic: Normal study.
MRI Hips and pelvic: Diffuse bone marrow reconversion and Presacral soft tissue mass as described, most likely extramedullary hematopoiesis.
So as per MRI the patient has extramedullary hematopoiesis presenting as soft tissue mass in the presacral area, from surgical point of view, the mass is not accessible, but still need to be excised. After that cervical and lumbar MRI spine was done to look if there are other extramodular hematopoietic sites and it showed:
Diffusely decreased T1 marrow signal intensity of the entire visualized bones which is in keeping with red marrow reconversion. Redemonstrations of a presacral well defined lobulated soft tissue mass measuring 5.3 x 3 cm, it is separated from the underlying sacrum, no sacral destruction. The mass is showing mild postcontrast enhancement suggestive of extramedullary hematopoiesis. Another similar smaller extradural mass measuring 2 cm is seen posterior to S1 vertebral body at the midline laterally displacing S1 traversing nerve roots.
Currently: she is still on blood transfusion to keep HB more than 10 in case of any surgical procedure was decided to be done, plan now is toward the excision of the mass. The patient is taking the following medications: Celecoxib 200 mg PRN, deferasirox 1080 mg oral daily, insulin therapy and vitamin D supplementation, Levothyroxine 75 mg daily, tramadol 50 mg oral BID.