HEMATOCASE

When winter is coming, you better keep warm

A 75-year-old patient presented with fluctuating swelling around the eyes, vasculitis at the lower legs and back of the upper legs and an extreme hypogammaglobulinaemia. An extensive work-up revealed the presence of secondary immunoglobulin M cryoglobulinaemia related to a monoclonal B-cell lymphocytosis. Precipitation of proteins also resulted in a decreased C1-esterase inhibitor causing angioedema. She was treated with an elderly chronic lymphocytic leukaemia regimen consisting of obinutuzumab and chlorambucil with a subsequent clinical and haematological remission.

(BELG J HEMATOL 2019;10(2):85–8)

Management of extended breast implant-associated anaplastic large cell lymphoma

SUMMARY

We report the case of a 69-year-old woman who presented an aggressive breast implant-associated anaplastic large cell lymphoma with supra- and infradiaphragmatic disease. The diagnosis was made 17 years after her first prosthesis, following a right breast carcinoma, and three years after the replacement of this first prosthesis. Breast implant-associated anaplastic large cell lymphoma is a rare form of non-Hodgkin lymphoma caused by a breast implant. Unique features of this case include the fast clinical extension of a lymphoma that is indolent in the vast majority of the cases. Indeed, less than two months after the first symptoms on the breast, cutaneous metastasis appeared on the right arm. The key diagnosis exams are histology and immunohistochemistry including CD30 and cytotoxic markers and a PET-scan to evaluate the extension of the disease. The treatment should include removal of the prosthesis and any associated mass. Local residual or unresectable disease may benefit from radiation therapy to the chest wall. For regional lymph node involvement or confirmed extended disease, adjuvant chemotherapy more in line with systemic anaplastic large cell lymphoma anaplastic lymphoma kinase-negative treatments is recommended. Finally, brentuximab vedotin, an anti-CD30 monoclonal antibody, showed encouraging results in refractory disease but still needs more prospective trials.

(BELG J HEMATOL 2018;9(7):279–84)

Blastic plasmacytoid dendritic cell neoplasm with skin, bone marrow involvement and transverse myelitis: a case report

SUMMARY

Blastic plasmacytoid dendritic cell neoplasm is a highly aggressive myeloid neoplasm with a high rate of central nervous system recurrence. We present a case to illustrate central nervous system involvement and possible treatment options.

(BELG J HEMATOL 2018;9(6):237–41)

Cyclic thrombocytopenia in a patient with polycythaemia vera: a case report

SUMMARY

We present a case of a 73-year old patient with polycythaemia vera in whom cyclic thrombocytopenia was diagnosed. Strong fluctuations in platelet count, ranging from 31 to 1334 × 10³/µL, were noticed after onset of hydroxyurea therapy. We did a literature search to find possible underlying causes of cyclic thrombocytopenia that could guide us towards a fast and appropriate diagnosis and an optimal treatment. In literature, provoked and unprovoked oscillations in platelet numbers have been described. Unprovoked oscillations can most likely be attributed to an unstable haematopoietic stem cell pool, as can be seen in polycythaemia vera. Provoked oscillations could be associated with myelosuppressive agents such as hydroxyurea. In both situations, a decrease in platelet count can be followed by a compensatory thrombopoietin-induced stimulation of megakaryocytes. Frequent hydroxyurea dose adjustments may be carried out in an attempt to control this cyclic pattern but, by contrast, may provoke a bouncing ball effect on platelet count. Certain patients will therefore benefit from maintaining therapy at a constant dose; while certain others require withholding or switching therapy. Cyclic thrombocytopenia is a rare finding and is frequently misdiagnosed as immune thrombocytopenia. If hydroxyurea-treated patients with a chronic myeloproliferative disorder present with thrombocytopenia, cyclic thrombocytopenia should be considered. Intensive follow-up with regular control of platelet count and personalised therapy is mandatory.

(BELG J HEMATOL 2018;9(5):188–91)

Refractory immune thrombocytopenic purpura in a child with vitamin D deficiency

SUMMARY

Immune thrombocytopenic purpura is an isolated thrombocytopenia consisting of premature platelet destruction mediated by self-reacting antibodies and an impaired platelet production. In children, most of the cases resolve spontaneously within six months. Several studies have shown a high incidence of vitamin D deficiency in auto-immune disorders, including immune thrombocytopenic purpura. We report the clinical history of an eight-year-old boy who presented with refractory immune thrombocytopenic purpura and major vitamin D deficiency. Supplementation in vitamin D was followed by a rapid normalisation of thrombocytosis. After six months of evolution, the child developed a relapse of immune thrombocytopenic purpura concomitant with a decrease of his serum level of vitamin D. Treatment with vitamin D associated with dapsone resulted in a prolonged remission. After thirteen months, dapsone treatment was stopped and the platelet count remained normal. This article reviews the approach of refractory immune thrombocytopenic purpura in children and discusses the potential interest of vitamin D in this disease.

(BELG J HEMATOL 2018;9(2):64–7.)

Successful treatment of immunoglobulin light chain amyloidosis with associated factor X deficiency

SUMMARY

Bleeding diathesis in light chain amyloidosis may be due to an acquired coagulation factor deficiency, most commonly factor X deficiency. This report describes the case of an elderly myeloma patient with associated light chain amyloidosis, nephrotic syndrome and factor X deficiency. Treatment with nine cycles of subcutaneous bortezomib + melphalan + prednisone resulted in a complete haematological remission with resolution of the nephrotic syndrome and normalisation of coagulation tests. This case report highlights the importance of performing coagulation screening tests in light chain amyloidosis. In addition, it illustrates that bortezomib-based regimens can induce rapid and complete haematological response with long-term correction of factor X levels.

(BELG J HEMATOL 2017;8(7):272–5)

Diffuse large B-cell lymphoma presenting as a spontaneous rupture of the spleen

SUMMARY

In this hematocase, a patient is presented with a spontaneous rupture of the spleen due to massive splenomegaly caused by a previously undiagnosed diffuse large B-cell lymphoma. Diagnosis and differentiation of this non-Hodgkin lymphoma is discussed with attention for the prognostic implications of the results. The association between spontaneous rupture of the spleen and haematological malignancies is further explored by means of earlier described cases. A take home message is given regarding this rare initial presentation of a lymphoma since immediate surgical intervention is imperative in these cases.

(BELG J HEMATOL 2017;8(6):239–43)