Indolent systemic mastocytosis associated with atypical small lymphocytic lymphoma: a rare form of concomitant lymphoproliferative disease

Systemic mastocytosis associated with cutaneous B-cell lymphoma

“…In myeloid malignancies associated with SM, neoplastic mast cells have the same clonal origin as malignant myeloid cells, but a relationship between neoplastic mast cells and coexisting lymphoid malignancy appears implausible. The assumption of two different clones is strongly supported by the fact that in our patient and in several other reported cases the KIT mutation was present in neoplastic mast cells but absent in neoplastic B cells . The rare co‐occurrence of SM and lymphoproliferative disorders could occur by chance and may not be causally related.…”

“…The majority of associated haematological malignancies are of myeloid origin, whereas an association of SM with lymphoproliferative disorders is rare. Apart from one case of hepatosplenic gamma‐delta T‐cell lymphoma with coexistent SM, associated lymphoproliferative disorders were solely B‐cell malignancies (Hodgkin, nodal non‐Hodgkin lymphoma, multiple myeloma, chronic lymphocytic leukaemia) . To the best of our knowledge, this is the first report of a patient with SM in association with cutaneous B‐cell lymphoma.…”

Simultaneous and Sequential Concurrent Myeloproliferative and Lymphoproliferative Neoplasms

“…SM) [35,36,45] or plasmacytoma (particularly CNL) [83,84,85,86,87,88,89,90,91]. Similar to classical Ph – MPN with B-CLL, in SM-B-CLL no common aberrant stem could be proven [35,36,45]. It has been speculated that, in a subfraction of CNL patients, the true neoplasm is the plasmacytoma while the neutrophilic proliferation is induced by plasmacytoma-derived cytokines [90].…”

“…Concurrent manifestation of two chronic-phase myeloid and lymphoid neoplasms in one patient is rare and occurs in approximately 1% of patients [11,12]. Several case reports and a few case series have addressed this issue [13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91]. Due to its rarity, there has been no systematic evaluation of which combinations of myeloid and lymphoid neoplasms are frequent/infrequent, and it is still a matter of debate whether two concurrent diseases in one patient are clonally related or represent independent aberrations.…”

A case report of systemic mastocytosis associated with multiple hematologic non–mast cell lineage diseases

“…Associated myeloid neoplasia accounts for 90% of SM‐AHNMD and includes myelodysplastic syndromes, myeloproliferative neoplasms, myelodysplastic/myeloproliferative syndromes, and acute myeloid leukemia (AML). SM associated with lymphoproliferative diseases is rare, and few cases have been described …”