Paraproteinaemia plus osteolytic lesions in typical hairy-cell leukaemia

Abstract

Most cases of hairy-cell leukaemia (HCL) involve proliferations of neoplastic B lymphocytes. In rare cases, M-proteins or osteolytic lesions have been documented in patients with HCL. In this study two patients with typical HCL are reported in whom both paraproteinaemia and osteolytic lesions of the femoral neck developed. In one of the patients the production of the M-protein by hairy cells could be established. In the other patient, at autopsy no signs of myeloma were found. The hairy cells from inside the osteolytic lesion had the same immunological phenotype as hairy cells from the peripheral blood, the spleen, and other parts of the bone marrow. These cases once more confirm the B-cell nature of many cases of HCL, and show that hairy cells can have functional capacities usually attributed to much more mature B lymphocytes, i.e. plasma cells.

Treatment options in skeletal localizations of hairy cell leukemia: A systematic review on the role of radiation therapy

“…Weh et al described 5 cases of bone localization in a series of 150 patients. The femoral neck was the most common site of involvement (4/5); 4 lesions were lytic, sometimes associated with pathological fractures, and [26] Left femoral neck None NS Died 1 months from BI 3 [26] Thoracic and lumbar vertebrae None Splen Alive at 3 months from BI 4 [26] Right femoral neck None NS Bone lesion at presentation; alive at 3 mo from BI 5 [26] Right femoral neck None NS Bone lesion at presentation; died 30 mo from BI 6 [21] Left femoral neck 60 Gy (30fr) VCR, PDN Alive at 18 mo from BI 7 [21] Left femoral neck 8 Gy (1 fr) NS Alive at 9 mo from first BI; developing multiple compression fractures of thoracic spine 8 [21] Parietal skull None NS -9 [27] Right femoral head 34,5 Gy (23fr) NS Left hip involvement treated with RT (24 Gy) 10 [2] Left femoral head 23 Gy PDN, Splen Relief of pain within 6 weeks Right femoral head and sacro-iliac region 22 Gy Normalization of bone by X-ray examination 11 [28] Right femoral neck 60 Gy (30fr) Splen Developed aseptic necrosis of right femour 1 year after first BI 12 [28] Right femoral neck, L2 None Splen, Chl Developed additional vertebral bodies collapse and rib involvement 4 mo after Chl 13 [29] Left humeral head 20 Gy Splen X-rays 2 mo after RT showed complete resolution of osteolysis; died of M. Kansasii dissemination 7 mo after BI 14 [29] Right femoral head 25 Gy (10 fr) Splen Alive at 6 years from BI 15 [29] Right femoral head None Splen, hip joint repl Developed iperdensity in left femoral head 2 years after first BI 16 [29] Several vertebral bodies None Splen, Dauno No complaints of osseus pain 12 mo after Dauno 17 [30] S-1 24 Gy None Concomitant right femoral osteolysis treated with total hip repl, alive at 14 mo from BI 18 [20] Right femoral neck 20 Gy (5 fr [21,29].…”

“…One of the most prominent clinical features is immunodepression with an increased risk of developing infections, involving mainly lung, urinary tract, liver, and CNS. Unusual complications are represented by intrathoracic and intra-abdominal lymphadenopathy [8], cutaneous involvement [9][10][11], pleural and peritoneal involvement [12], ocular [13], meningeal [14], renal [15], hernial sac localizations [16], amyloidosis [17], and paraproteinemias [3,[18][19][20].…”

Isolated sacral injury in hairy cell leukemia: A case report and analysis of literature data

“…Диагноз специфического поражения кости при ВКЛ необ-ходимо подтверждать гистологическим исследованием биопта-та, а так как материал, получаемый при игольной биопсии, ча-сто представлен только некротической тканью, для окончатель-ной диагностики может быть необходима резекция пораженно-го участка кости. Гистологическое исследование биоптата по-зволяет установить специфический характер поражения и про-вести дифференциальный диагноз с инфекционным (в частно-сти, туберкулезным) поражением, некрозом, метастазом другой опухоли, множественной миеломой (описано как сочетание ВКЛ и миеломы, так и последовательное возникновение этих заболе-ваний) [19][20][21].…”

Hairy cell leukemia: biology, clinical diagnosis, unusual manifestations and associated disorders

“…In rare cases, there may be an associated large granular lymphocytosis or an increase in NK cells (124). An increase in CD3 positive γδ lymphocytes may also be detected in these cases.…”