Case 151 – Update 1

Thanks a lot for your responses.

Fevers continue despite good antibiotic cover. All cultures are sterile. Lymphadenopathy persists and he has ongoing splenomegaly.

Peripheral blood is sent for flow cytometry. There is only 1% of CD 45 weak population positive for CD 34 , HLA-DR and CD 117. Other markers are negative. CD14 positive monocytes comprise 72% of the nucleated population.

Platelet count keeps dropping and he now requires daily platelet transfusions over the last 72 hours.

Viruses including EBV/CMV/Adenovirus and TORCH panel are all negative. HbF percentage is 8.2% which is normal for age. LDH is 892 and rising , Clauss Fibrinogen is 1.2 g/L and Ferritin is 9500 mcg/L.

Ultrasound of the abdomen shows enlarged liver with splenic parenchymal infiltrates. A CT TAP does not aid apart from confirming cervical lymphadenopathy and splenic infiltrates.

He is wheeled into theatres for a bone marrow under GA. Aspirate is aparticulate and there is left shifted granulopoiesis. There is dyserythropoiesis along with basophilia and prominent promonocytes and monocytes. Blast population is 2%. No morphological evidence of acute leukemia.

The flow again shows only 4% of CD 45 weak nucleated cell population. Similar markers as the peripheral blood. Trephine is reported as : Disorganised marrow with a preponderance to left shifted
granulopoiesis. Monocytes mildly increased. Megakaryocytes and erythroid cells
are significantly reduced.

Samples from bone marrow for infectious pathogens including AFB are negative.

Karyotype is 46 XY , FISH is negative for BCR ABL and molecular studies are awaited

  • Is there a component of HLH here ? Is it primary or secondary to an underlying problem ?
  • Given the background and age – what further tests does would help us narrow down our differentials?
  • What specific molecular tests from the bone marrow would be helpful?

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