https://orcid.org/0000-0002-8640-9776
![A 44-year-old man was referred in our hospital for asthenia, digestive disorders, faintness with fall at home. A computed tomography scan revealed a severe ruptured spleen, which immediately led to splenectomy. At this time, the blood count showed high leukocytosis (290 × 109/L) and anemia (8 g/dL) without thrombocytopenia (170 × 109/L). Blood smear examination revealed 96% of small blast cells with a high nuclear/cytoplasmic ratio and irregular nucleus (panel A; May-Grünwald-Giemsa stain, original magnification ×500). Flow cytometry detected CD45dim/cytCD3+/sCD3−/CD34−/TdT+/CD1a+/CD2+/CD5+/CD7+/CD4−/CD8+ T lymphoblasts (panel B). Surprisingly, the white blood cell count decreased drastically without treatment to 3 × 109/L 2 days later. The blast proportion also decreased to 1% with a concomitant high cell lysis rate confirmed by elevated lactate dehydrogenase (7830 U/L), suggesting that the spleen was the main source of blood blast cell dissemination. Histology confirmed massive infiltration of the spleen by cortical lymphoblastic T cells (panel Ci, hematoxylin and eosin stain [HES]; panel Cii, immunohistochemistry [IHC]). Due to an unsuccessful aspiration, bone marrow infiltration was not assessed. Molecular analysis demonstrated an unfavorable prognosis with T-cell acute lymphoblastic leukemia (T-ALL) classifier (NOTCH/FBXW7/RAS wild-type, PTEN mutation and deletion). Eleven days after splenectomy, induction chemotherapy by Group of Research on Adult ALL (GRAALL) protocol was started, which allowed a complete remission.](https://ash.silverchair-cdn.com/ash/content_public/journal/blood/136/1/10.1182_blood.2020005728/4/m_bloodbld2020005728f1.png?Expires=1767713707&Signature=zQQOXPDnxods0DwBUff9lvQAUroLUBG-bKKFgHO0rAthdrHMzo6zbBHkFREKUWXE5Q6NWP4XGGlIwLgwogcZrJ9PD8xeA51JdU73MiWVfAPg4-iWGk9GOrm5KDkrL2OMpZetgtgHRjAJyCb1RHsLl8ONlFVr0pMCVpRoDGqe6gs52TGtq90VkpA7as2nUzZlJRK0tdAeDBCV38hBKsWsmrQjERX75QU8uU2erm43obcqYl5GZM419FO4VJQccI822t4BVxJgi5t195qRzYsYhe9Z8QBF3zMSFfVkyc5C8vFCJ5wA5ajELuSdYB2KAnw08Cm0f-euUjVFjhE9xY1PXQ__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
A 44-year-old man was referred in our hospital for asthenia, digestive disorders, faintness with fall at home. A computed tomography scan revealed a severe ruptured spleen, which immediately led to splenectomy. At this time, the blood count showed high leukocytosis (290 × 109/L) and anemia (8 g/dL) without thrombocytopenia (170 × 109/L). Blood smear examination revealed 96% of small blast cells with a high nuclear/cytoplasmic ratio and irregular nucleus (panel A; May-Grünwald-Giemsa stain, original magnification ×500). Flow cytometry detected CD45dim/cytCD3+/sCD3−/CD34−/TdT+/CD1a+/CD2+/CD5+/CD7+/CD4−/CD8+ T lymphoblasts (panel B). Surprisingly, the white blood cell count decreased drastically without treatment to 3 × 109/L 2 days later. The blast proportion also decreased to 1% with a concomitant high cell lysis rate confirmed by elevated lactate dehydrogenase (7830 U/L), suggesting that the spleen was the main source of blood blast cell dissemination. Histology confirmed massive infiltration of the spleen by cortical lymphoblastic T cells (panel Ci, hematoxylin and eosin stain [HES]; panel Cii, immunohistochemistry [IHC]). Due to an unsuccessful aspiration, bone marrow infiltration was not assessed. Molecular analysis demonstrated an unfavorable prognosis with T-cell acute lymphoblastic leukemia (T-ALL) classifier (NOTCH/FBXW7/RAS wild-type, PTEN mutation and deletion). Eleven days after splenectomy, induction chemotherapy by Group of Research on Adult ALL (GRAALL) protocol was started, which allowed a complete remission.
This case reveals a very unusual primary splenic T-lymphoblastic leukemia/lymphoma with a spontaneous blast cell decrease before initial treatment, highlighting a very rare blast clearance following a ruptured pathologic spleen.
A 44-year-old man was referred in our hospital for asthenia, digestive disorders, faintness with fall at home. A computed tomography scan revealed a severe ruptured spleen, which immediately led to splenectomy. At this time, the blood count showed high leukocytosis (290 × 109/L) and anemia (8 g/dL) without thrombocytopenia (170 × 109/L). Blood smear examination revealed 96% of small blast cells with a high nuclear/cytoplasmic ratio and irregular nucleus (panel A; May-Grünwald-Giemsa stain, original magnification ×500). Flow cytometry detected CD45dim/cytCD3+/sCD3−/CD34−/TdT+/CD1a+/CD2+/CD5+/CD7+/CD4−/CD8+ T lymphoblasts (panel B). Surprisingly, the white blood cell count decreased drastically without treatment to 3 × 109/L 2 days later. The blast proportion also decreased to 1% with a concomitant high cell lysis rate confirmed by elevated lactate dehydrogenase (7830 U/L), suggesting that the spleen was the main source of blood blast cell dissemination. Histology confirmed massive infiltration of the spleen by cortical lymphoblastic T cells (panel Ci, hematoxylin and eosin stain [HES]; panel Cii, immunohistochemistry [IHC]). Due to an unsuccessful aspiration, bone marrow infiltration was not assessed. Molecular analysis demonstrated an unfavorable prognosis with T-cell acute lymphoblastic leukemia (T-ALL) classifier (NOTCH/FBXW7/RAS wild-type, PTEN mutation and deletion). Eleven days after splenectomy, induction chemotherapy by Group of Research on Adult ALL (GRAALL) protocol was started, which allowed a complete remission.
This case reveals a very unusual primary splenic T-lymphoblastic leukemia/lymphoma with a spontaneous blast cell decrease before initial treatment, highlighting a very rare blast clearance following a ruptured pathologic spleen.
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