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Bone marrow findings in lupus patients with pancytopenia
15
HEMATOLOGY
INTRANUCLEAR INCLUSIONS IN MYELOMA CELLS IN A CASE OF NONSECRElORY MULlIPLE MYELOMA C.S.Feng. Haematology Laboratory and Blood Bank, Prince of Wales Hospital, Shatin, N.T., Hong Kong Inclusions in the nucleus, compared to those in the cytoplasm, are rare in myeloma cells but have been reported in all electrophoretic varieties of multiple myeloma except the nonsecret.ory type. In this unusual case, a 54 year-old Chinese woman had a pathological fracture of the left femur and biopsy of the fracture site revealed a round cell tumor conipatib!e with plasmacytoma. A bone marrow aspirate revealed 50% plasma cells, many of which contained intranuclear inclusions. Protein electrophoresis was normal with no paraprotein and urine was free from Bence Jones protein. Under electron microscopy, the plasma cells showed electron-dense spherules not circumscribed by a membrane. The absence of a membrane was unusual because according to all reported cases, these intranuclear inclusions were invariably membrane-bound. The association of nonsecretion o f paraprotein in multiple myeloma which is rare, and the absence of membrane enclosing these intranuclear inclusions which is heretofore unreported, i s probably not co-incidental but causally related. In this instance, the paraprotein which was produced in the nucleus arid stored in the form of intranuclear inclusions, failed to be detected in serum and urine because of non-interaction between these inclusions and membranes of the nucleus and endoplasmic reticulum.
GELATINOUS TRANSFORMATION OF BONE MARROW IN SYSTEMIC LUPUS ERYTHEMATOSUS. Chi-Shun Feng, Margaret H.L. Ng, Edmund K. Li, Department o f Morbid Anatomy, Prince of Wales Hospital' Shdtln, N.T., Hong Kong. Gelatinous transformation is characterized by disruption of normal architecture of bone marrow in that the marrow cells arrange themselves in long cords, small clusters, and singly; and fat cells are partially replaced by a fibrillary substance which stains positive with Alcian Blue alone but negative with Alcian Blue if pre-treated by hyaluronidase. Gelatinous transformation of marrow is a rare entity described in cachexia, anorexia nervosa, and myxedema. Its association with systemic lupus erythematosus has never been reported. We found this condition in 3 out of 30 lupus patients with pancytopenia. Two o f the patients were cachetic, one o f whom also had active tuberculosis. We propose Chat gelatinous transformation of marrow can be associated with systemic lupus, and that its detection in lupus patients calls for an evaluation o f nutritionai status, and a search f o r chronic debilitating infections such as tuberculosis. Presenter: Dr. Margaret Ng, Haematology Laboratory Prince of Wales Hospital, Hong Kong.
Presenter: Dr. Chi-Shun Feng, PWH, Hong Kong.
SUPERNATANT I(+ AND IN VITRO HAEMOLYSIS OF WASHED RE0 BLOOD CELLS STORED IN SALINE FOR 3 DAYS. C.S.Feng. S.S.Tsang. Haematology Laboratory and Blood Bank Prince of Wales Hospital, Shatin, N.T.. Hong Kong. Washed Red Blood Cells (WRBC) is allowed a shelf-life of 24 hours. This study 1 s undertaken to determine whether 1 ) the K+ level in the supernatant at 24 hours is acceptably-low f o r neonatal transfusion, and 2) the preservation of red cells in saline, as measured by in vitro haemolysis can be extended to beyond 24 hours. 22 units of donor blood o f various storage age washed manually, and 4 units washeo by Haemonetics were studied. jdmples takeil from each were divided into aliquots and tested for K + level (mmol/l) and I loss of haemoglobin (Hb) in the supernatant at 6, 24, 48 and 72 hours. Results were as follows: red cells hours of storage 6 24 48 72 age (n) Manual wash 4-7 days 9.2 13.1 K 0.7 4.8 2.1 2.5 1.4 1.7 Hg (7) 14 days K 0.8 4.2 7.6 10.6 (7) Hg 2.4 3.3 4.0 4.3 21 days K 0.9 4.0 6.5 8.0 4.6 5.0 H9 2.6 4.0 (8) Haemonetics 6-12 days K 0.2 0.8 3.9 / (4) Hg 1.3 2.6 3.8 I Although the supernatant K+ was acceptably low, in vitro haemolysis was excessive compared to blood stored in standard preservatives. We propose in vivo survival study for WRBC after 24 hours of storage in saline; alternative measures such as additives to WRBC immediate postwash f o r better preservation. Presenter: Sophia Tsang, Blood Bank, PWt-1, Hong Kong
BONE MARROW FINDINGS IN LUPUS PATIENTS W I T H PANCYTOPENIA. C.S.Fenq, M.H.L.Nq. S.C.Szeto, E.K.Li. Department o f Morbid Anatomy, Prince of Wales Hospital, Shatin, N.T., Honq Konq. The marrow findinas o f 23 lupus patients with pancytopenia were reported. The most commnn findings were dyserythropoiesis and hypoplasia, both occurrinq in 9/23 (39%) of the cases. Neither feature was definitiveIy related t o cytotoxic drug therapy since some cases were only Created by steroids and two cases were never treated. 3 o f the hypoplastic marrows also had gelatinous transformation, a condition characterized by disruption o f marrow architecture, fat atrophy, and deposition of hyaluronic acid. Another common finding was lymphocytosis which occurred in 5/23 (22%) of the cases, 2 o f which also had associated plasrnacytosis. 2 cases were associated with hyperplastic marrow (cellularity >70%),indicatinq peripheral destruction o f blood cells and cornpensat.ory marrow hyperplasia. Reports in the literature on bone marrows in systemic lupus erythematosus were conflictinq, describinq mainly hypoplasia, vasculitis, and plasmacytosis. In o u r series, we described hypoplasia and lymphocytosis/plasmacytos~s~ in addition we described findings heretofore unreported: gelatinous transformation, dyserthropoeisis, and marrow hyperplasia. Presenter: Dr. Chi-Shun Fens,
PWH,
Hong Konq
HEMATOLOGY
INTRANUCLEAR INCLUSIONS IN MYELOMA CELLS IN A CASE OF NONSECRElORY MULlIPLE MYELOMA C.S.Feng. Haematology Laboratory and Blood Bank, Prince of Wales Hospital, Shatin, N.T., Hong Kong Inclusions in the nucleus, compared to those in the cytoplasm, are rare in myeloma cells but have been reported in all electrophoretic varieties of multiple myeloma except the nonsecret.ory type. In this unusual case, a 54 year-old Chinese woman had a pathological fracture of the left femur and biopsy of the fracture site revealed a round cell tumor conipatib!e with plasmacytoma. A bone marrow aspirate revealed 50% plasma cells, many of which contained intranuclear inclusions. Protein electrophoresis was normal with no paraprotein and urine was free from Bence Jones protein. Under electron microscopy, the plasma cells showed electron-dense spherules not circumscribed by a membrane. The absence of a membrane was unusual because according to all reported cases, these intranuclear inclusions were invariably membrane-bound. The association of nonsecretion o f paraprotein in multiple myeloma which is rare, and the absence of membrane enclosing these intranuclear inclusions which is heretofore unreported, i s probably not co-incidental but causally related. In this instance, the paraprotein which was produced in the nucleus arid stored in the form of intranuclear inclusions, failed to be detected in serum and urine because of non-interaction between these inclusions and membranes of the nucleus and endoplasmic reticulum.
GELATINOUS TRANSFORMATION OF BONE MARROW IN SYSTEMIC LUPUS ERYTHEMATOSUS. Chi-Shun Feng, Margaret H.L. Ng, Edmund K. Li, Department o f Morbid Anatomy, Prince of Wales Hospital' Shdtln, N.T., Hong Kong. Gelatinous transformation is characterized by disruption of normal architecture of bone marrow in that the marrow cells arrange themselves in long cords, small clusters, and singly; and fat cells are partially replaced by a fibrillary substance which stains positive with Alcian Blue alone but negative with Alcian Blue if pre-treated by hyaluronidase. Gelatinous transformation of marrow is a rare entity described in cachexia, anorexia nervosa, and myxedema. Its association with systemic lupus erythematosus has never been reported. We found this condition in 3 out of 30 lupus patients with pancytopenia. Two o f the patients were cachetic, one o f whom also had active tuberculosis. We propose Chat gelatinous transformation of marrow can be associated with systemic lupus, and that its detection in lupus patients calls for an evaluation o f nutritionai status, and a search f o r chronic debilitating infections such as tuberculosis. Presenter: Dr. Margaret Ng, Haematology Laboratory Prince of Wales Hospital, Hong Kong.
Presenter: Dr. Chi-Shun Feng, PWH, Hong Kong.
SUPERNATANT I(+ AND IN VITRO HAEMOLYSIS OF WASHED RE0 BLOOD CELLS STORED IN SALINE FOR 3 DAYS. C.S.Feng. S.S.Tsang. Haematology Laboratory and Blood Bank Prince of Wales Hospital, Shatin, N.T.. Hong Kong. Washed Red Blood Cells (WRBC) is allowed a shelf-life of 24 hours. This study 1 s undertaken to determine whether 1 ) the K+ level in the supernatant at 24 hours is acceptably-low f o r neonatal transfusion, and 2) the preservation of red cells in saline, as measured by in vitro haemolysis can be extended to beyond 24 hours. 22 units of donor blood o f various storage age washed manually, and 4 units washeo by Haemonetics were studied. jdmples takeil from each were divided into aliquots and tested for K + level (mmol/l) and I loss of haemoglobin (Hb) in the supernatant at 6, 24, 48 and 72 hours. Results were as follows: red cells hours of storage 6 24 48 72 age (n) Manual wash 4-7 days 9.2 13.1 K 0.7 4.8 2.1 2.5 1.4 1.7 Hg (7) 14 days K 0.8 4.2 7.6 10.6 (7) Hg 2.4 3.3 4.0 4.3 21 days K 0.9 4.0 6.5 8.0 4.6 5.0 H9 2.6 4.0 (8) Haemonetics 6-12 days K 0.2 0.8 3.9 / (4) Hg 1.3 2.6 3.8 I Although the supernatant K+ was acceptably low, in vitro haemolysis was excessive compared to blood stored in standard preservatives. We propose in vivo survival study for WRBC after 24 hours of storage in saline; alternative measures such as additives to WRBC immediate postwash f o r better preservation. Presenter: Sophia Tsang, Blood Bank, PWt-1, Hong Kong
BONE MARROW FINDINGS IN LUPUS PATIENTS W I T H PANCYTOPENIA. C.S.Fenq, M.H.L.Nq. S.C.Szeto, E.K.Li. Department o f Morbid Anatomy, Prince of Wales Hospital, Shatin, N.T., Honq Konq. The marrow findinas o f 23 lupus patients with pancytopenia were reported. The most commnn findings were dyserythropoiesis and hypoplasia, both occurrinq in 9/23 (39%) of the cases. Neither feature was definitiveIy related t o cytotoxic drug therapy since some cases were only Created by steroids and two cases were never treated. 3 o f the hypoplastic marrows also had gelatinous transformation, a condition characterized by disruption o f marrow architecture, fat atrophy, and deposition of hyaluronic acid. Another common finding was lymphocytosis which occurred in 5/23 (22%) of the cases, 2 o f which also had associated plasrnacytosis. 2 cases were associated with hyperplastic marrow (cellularity >70%),indicatinq peripheral destruction o f blood cells and cornpensat.ory marrow hyperplasia. Reports in the literature on bone marrows in systemic lupus erythematosus were conflictinq, describinq mainly hypoplasia, vasculitis, and plasmacytosis. In o u r series, we described hypoplasia and lymphocytosis/plasmacytos~s~ in addition we described findings heretofore unreported: gelatinous transformation, dyserthropoeisis, and marrow hyperplasia. Presenter: Dr. Chi-Shun Fens,
PWH,
Hong Konq