I. Vrelust MD

Case series of two patients with KIT D816V positive systemic mastocytosis with associated chronic myelomonocytic leukaemia

SUMMARY

In this case report, we describe two patients with systemic mastocytosis with an associated haematological neoplasm. The KIT c.2447A>T;p. (Asp816Val) (D816V) mutation, the original driver mutation of mastocytosis, can, in combination with additional genetic abnormalities, drive the clonal evolution towards an additional myelodysplastic or myeloproliferative neoplasia. When patients present with a dominant phenotype of the latter neoplasia, which is often the cause in chronic myelomonocytic leukaemia (CMML) or acute myeloid leukaemia (AML), the original mastocytosis could be overlooked, missing therapeutic opportunities.

(BELG J HEMATOL 2023;14(4):178–82)

Intracranial myeloid sarcoma as rare presentation of BCR-ABL-positive acute myeloid leukaemia: A case report

SUMMARY

Introduction: BCR-ABL positive acute myeloid leukaemia is recognised as a provisional entity in the WHO 2016 classification for AML and related precursor neoplasms. Myeloid sarcoma is an extramedullary accumulation of immature myeloid cells, intracranial occurrence is rare.

Case report: A 40-year old male presented with an intracranial mass and peripheral blood blastosis of 65%. A diagnosis of acute myeloid leukaemia with intracranial myeloid sarcoma was presumed, requiring urgent cranial decompression. The patient received standard AML induction therapy with daunorubicine and cytarabine. Molecular analysis surprisingly revealed BCR-ABL positivity.

Discussion: BCR-ABL positive AML is a rare entity and needs to be differentiated from CML in blast crisis because of therapeutic consequences. In our case, clinical and molecular features suggested a diagnosis of AML.

Conclusion: To the best of our knowledge, this is the first case of intracranial myeloid sarcoma as a concurrent presentation of BCR-ABL-positive acute myeloid leukaemia.

(BELG J HEMATOL 2021;12(4):165-8)

Systemic capillary leak syndrome as a prodrome of extranodal natural killer (NK)/T-cell lymphoma

Summary

We present the case of a 58-year-old male patient with a long-standing, intermittent oedema of the lower extremities and significant spontaneous variations in haematocrit values. Repeated examinations failed to reveal a clear etiology until the patient suffered from a severely painful exacerbation of leg oedema and hypotension. Laboratory analysis showed hypoalbuminemia. The combination of oedema, hypotension, hypoalbuminemia and hemoconcentration was indicative of a systemic capillary leak syndrome. This condition is known to be associated with monoclonal gammopathy, as was the case in our patient. New investigations showed suspicious lesions in the nasopharynx, scrotum and breast. Biopsies of this breast mass as well as bone marrow biopsy showed the presence of an extranodal natural killer/T-cell lymphoma, nasal type. Polychemotherapy was administered according to the SMILE schedule leading to a remission after two cycles. The patient then underwent autologous hematopoietic stem cell transplantation. The patient is currently without signs of systemic capillary leak syndrome. This report illustrates that systemic capillary leak syndrome may occur as a prodrome of haematological malignancies, such as natural killer/T-cell lymphoma and documents that it is responsive to chemotherapy.

(BELG J HEMATOL 2014;5(4):148–53)

P3.05 Efficacy of azacitidine in Belgian patients: results of a real-life non-interventional, post-marketing survey

Myeloid sarcoma or lymphoblastic lymphoma? A closer look at the laboratory diagnosis

Summary

Although a myeloid sarcoma is a rare form of extramedullary leukaemia, its early diagnosis has been proven to be of utmost importance. Its presence is strongly related to the onset or the presence of systemic bone marrow leukaemia. However, the diagnosis of myeloid sarcoma is not straightforward. In the existing literature, approximately half of the cases of myeloid sarcoma were initially misdiagnosed as lymphoma. The current case reports details on the laboratory diagnosis of myeloid sarcoma in a 25-year old male. The laboratory presentation of myeloid sarcoma and the consecutive steps in order to correctly diagnose myeloid sarcoma using a variety of laboratory techniques including microscopy, flow cytometry and cytogenetics are highlighted.

(BELG J HEMATOL 2013;4(3): 106–109)

P.17 Complete responses of the TEMPI syndrome to bortezomib

P.20 Primary Intraocular Lymphoma: a Case Report