An interesting rare case of hematological malignancy!
54 years old male patient not a known case of any chronic illness presented with:
(1)Lump on left side of upper abdomen since 18 months (Firm mass, around the size of a fist initially which gradually increased in size;Associated with dragging, mild continuous abdominal discomfort, easy satiety after meals and abdominal fullness),
(2)Generalised body weakness and easy fatiguability since 1 year (Patient used to take rest after some work,History of malaise and dull boring continous pain in the lower limbs,more during night time),
(3)Bleeding gums and petechial spots over the body since 2 months(fresh red blood even without trauma but was more after brushing the teeth.Bleeding was continous and stopped after pressing the oozing site with cotton.History of spontaneous petechial spots present over body, more on upper and lower limbs, which slowly fade with time and new spots occurred at another site.)
(4)History of decreased appetite associated with weight loss present.
Past History- 3 units of blood transfusion one year back.
Also,18 months back was admitted for anemia. 2 units PCV were transfused and bone marrow biopsy was done.
No past history of bleeding disorder/hypertension.
Patient-conscious and oriented.
Oral cavity revealed poor oral hygiene with one bleeding spot present on the right 2nd upper molar. No white patches in oral cavity were present.
Petechial spots present on the lateral wall of the axilla,anterior surface of right arm,shin of tibia of bilateral lower limbs and few petechial spots over the abdomen.
Globular abdomen, with abdominal fullness present over the left upper abdomen, normal slit umblicus.
Liver palpable 4 cm below the right costal margins in mid-clavicular line.Upper border of liver present in 6th intercostal space in mid clavicular line, firm, smooth, non tender, moving normally with respiration.Liver span 17cm.
Spleen is palpable 22 cm below the left costal margin towards the umblicus, non tender, firm, smooth, notch palpable, moving normally with respiration.Medially also the spleen crossed the midline 4 cm to the right of the umblicus.
Immunophenotyping from peripheral blood:
CD 45–Positive;HLA DR-Positive;CD 19-Positive;CD 20-positive;CD 22-Positive;LAMBDA-Positive. (Light chain);CD11c-Positive;CD 25-Positive;CD 123-Positive;CD 103–Positive;cyCD22 and cyCD 79a–Positive.
Give the complete diagnosis.
What could be differential diagnosis in this condition?
What would be appropriate line of management?
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