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. 2022 Aug 9;6(15):4501-4505.
doi: 10.1182/bloodadvances.2022007450.

Treatment of an HLH-mimic disease based on HAVCR2 variants with absent TIM-3 expression

Samantha A M Tromp  1   2 , Marijn A Gillissen  3 , Sophie J Bernelot Moens  3 , Ester M M van Leeuwen  2 , Machiel H Jansen  2 , Lianne Koens  4 , Caroline E Rutten  3 , Taco W Kuijpers  1   2
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Treatment of an HLH-mimic disease based on HAVCR2 variants with absent TIM-3 expression

Samantha A M Tromp et al. Blood Adv. .
No abstract available

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Graphical abstract
Figure 1
Figure 1
Clinical and immunological findings. (A) Absolute platelet numbers and hemoglobin levels over time (from acute phase until the present time). (B) FDG-PET showing hepatosplenomegaly and extensive mesenteric fat infiltration. (C) CD4/CD8 double-stained omental biopsy specimen (original magnification ×40), with CD4+ lymphocytes stained brown and CD8+ lymphocytes stained red, infiltrating between the mature adipocytes. (D) Hematoxylin and eosin–stained bone marrow biopsy specimen (original magnification ×40), showing trilineage hematopoiesis and scattered histiocytic cells with packed erythrocytes in the cytoplasm. Inset in the left bottom corner is Giemsa-stained bone marrow aspirate (original magnification ×100), highlighting a hemophagocytic histiocyte. (E) NK-cell function before (left) and after (right) treatment. (F) Absolute B-cell, T-cell, and NK-cell counts over time (from acute phase until the present time). Green shading indicates age-dependent normal values.
Figure 2
Figure 2
TIM-3 expression and upregulation in vitro. (A) Ex vivo TIM-3 expression on day 0: HD (red); patient (blue). (B) Representative histogram of TIM-3 expression on day 0. (C) TIM-3–expressing CD4 T-cells after 3 days of culture: HD (red); patient (blue). (D) TIM-3–expressing CD8 T-cells after 3 days of culture: HD (red); patient (blue). (E) TIM-3–expressing NK-cells after 3 days of culture: HD (red); patient (blue). (F) Representative histogram of TIM-3 expression after 3 days of culture.
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References

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