Gastric lymphoma causing granulocytopenia and cold intolerance, with recovery after treatment

Abstract

A 34-year-old man was found to have granulocytopenia with a white blood count of 2.3 x 10(9) l-1, consisting of 10% segmented neutrophils, 50% monocytes and 40% lymphocytes. A bone marrow aspirate showed 20% promyelocytes and 10% blasts with monoblastic features, and a smouldering myelomonocytic leukaemia was considered to be a possible diagnosis. In cold weather the patient experienced cold intolerance with acrocyanosis and small ulcerations on the ears. The test for heparin-precipitable protein ('cryofibrinogen') was strongly positive. During the following year, these signs and symptoms persisted, and the patient also developed constant moderate pain in the epigastric region. Gastroscopy revealed a large lymphoma of the stomach, which was a high-grade malignant centroblastic type of non-Hodgkin's lymphoma. After successful removal of the tumour, and six courses of potent cytostatic combinations, the patient recovered completely, and the granulocytopenia and cold intolerance disappeared.

Acrocyanosis: The Flying Dutchman

“…32,153 Multiple reports describe an association between acrocyanosis and cold agglutinins, cryoglubulins, or cryofibrinogen. 28,33,154–156 Almost a third of all patients with positive antiphospholipid antibodies have acrocyanosis. 30,40,157,158 Anti-cardiolipin antibodies have been reported in patients with acrocyanosis.…”

“…Lymphoproliferative [31][32][33][34] Myeloproliferative disorders [35][36][37] Cold agglutinins 28,38,39 Ethylmalonic aciduria [59][60][61] Cytochrome C oxidase deficiency 62,63 Mitochondrial disease (oxidative phosphorylation disorders) 64 Spondyloenchondrodysplasia 65 Palmoplantar keratoderma syndrome 66 Fucosidosis 67 Down's syndrome 68 Prader-Willi syndrome 69 Sneddon's syndrome 70,71 Aicardi-Goutières syndrome 72,73 Marfan's disease Riley-Day syndrome (familial dysautonomia) 74 Ehlers-Danlos syndrome 75,76 Psychiatric:…”

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“…6,7,9,15 In addition to being the first reported case after a liver transplant, we also believe this is the first reported association between neuroendocrine tumor and CF. A previous study by Belizna and associates 14 recommended a systematic search for lymphoma be performed in patients with an established diagnosis of essential CF, as 27% of patients diagnosed with essential CF are found to have lymphoma in a few years.…”

“…28 In addition, patients with CF secondary to malignancy have been shown to respond to tumor resection. 15 However, treatment approaches are based on cases reports and small case series.…”

Cryofibrinogenaemia: not just skin deep

“…Treatment of primary cryofibrinogenaemia is usually less satisfying, whereas treatment of secondary cryofibrinogenaemia consists of managing the underlying condition as well 1 12. Outcomes for such approaches, however, are limited to individual or small series of cases, making generalised statements regarding treatment difficult.…”