Chi-Shun Feng

Bone Marrow Findings in Lupus Patients with Pancytopenia

&NA; The marrow findings of 23 lupus patients with pancytopenia were reported. The most common findings were dyserythropoiesis and hypoplasia, both occurring in 9/23 (39%) of the cases. Neither feature was definitely related to cytotoxic drug therapy since most cases were treated by steroids only. Three of the hypoplastic marrows also showed gelatinous transformation, a condition characterized by disruption of marrow architecture, fat atrophy, and deposition of hyaluronic acid. Another common finding was lymphocytosis which occurred in 5/23 (22%) of the cases, 2 of which also had associated plasmacytosis. Two cases were associated with hyperplastic marrow, indicating peripheral destruction of blood cells and compensatory marrow hyperplasia. Reports in the literature on bone marrows in systemic lupus erythematosus are conflicting, describing mainly hypoplasia, vasculitis, plasmacytosis, red cell aplasia, and myelofibrosis. In our series, we found hypoplasia and lymphocytosis/plasmacytosis; in addition we described findings previously unreported: gelatinous transformation, dyserythropoiesis, and marrow hyperplasia.

A variant of gelatinous transformation of marrow in leukemic patients post-chemotherapy

&NA; A variant of gelatinous transformation of marrow was described in leukemic patients post‐chemotherapy. This lesion was found in 8 out of 1833 post‐chemotherapy bone marrows from 429 patients with leukemia. Histologically, this variant form was identical to the classical gelatinous transformation except for the absence of fat atrophy. In marrow smears, mucoid strands were seen between marrow particles and oriented along the direction of spread. In clot and trephine sections, an eosinophilic ground substance was widespread, filling out spaces around the fat cells, which occupied the normal proportion of marrow space. At high magnification, this ground substance appeared granular and fibrillary, a non‐specific feature which could also be seen in fibrin clot commonly found in marrow sections. This eosinophilic ground substance, however, can be distinguished from fibrin clots by positive staining with Alcian Blue (pH 2.3) and inhibition of the staining after treatment with hyaluronidase. Clinically, in contrast to the classical form, this variant form of gelatinous transformation was acute in onset, transient, and associated with chemotherapy but not with cachexia.

An analysis of donor blood wastage due to outdating in a large teaching hospital

Summary We analyzed the pattern and rate of donor blood outdating in our hospital from 1986 to 1990. We found that there was a sharp drop in blood outdating since the implementation of the Type and Screen crossmatch protocol. The outdating rate was reduced from 11.5% to 1.3% for whole blood and from 4.9% to 0.4% for red cells. In absolute numbers, wastage of blood due to outdating was cut from 2,570 units in 1986–87 (a 2 yr period before Type and Screen) to only 227 units in 1988–89 (a 2 yr period after Type and Screen). We also found that the outdating rate varied among different blood groups (group 0 < A = B < AB), and the outdating of whole blood was consistently more than red cells throughout the study period. The results of our utilization review were conveyed to local hospitals and the blood supplier in an effort to preserved donor blood.

Evaluation of laboratory tests for lupus anticoagulant in a group of Chinese lupus patients

&NA; The sera of 69 Chinese patients with systemic lupus erythematosus were tested for the presence of lupus anticoagulant (LA) by a panel of laboratory tests: Kaolin clotting time (KCT), dilute Russell viper venom time (DRVVT) and platelet neutralization procedure (PNP). The prevalence of LA varied among the 3 tests (10–19%), and was 10% when LA was considered present if either KCT or DRVVT and the PNP were positive. Concordance was fair between KCT and PNP, but was poor for DRVVT with either of the other 2 tests. Only 2 of our lupus patients had a history of thrombo‐embolic disease, and neither were serologically positive for LA. The incidence of thrombo‐embolic diseases and that of LA were both too low in this group of Chinese lupus patients for their association to be evaluated.

A morphologic variant of May–Hegglin anomaly in a Chinese girl

&NA; An 8 yr old Chinese girl was investigated for easy bruising and mild thrombocytopenia. Platelet aggregation studies and coagulation tests were found to be normal. The giant platelets and Döhle‐like cytoplasmic inclusions in granulocytes confirmed the diagnosis of May‐Hegglin anomaly. The fathers granulocytes also had Döhle‐like inclusions and one paternal aunt had a history of bleeding tendency. Review of literature showed that such Döhle‐like inclusions had always been described morphologically as crescentic or spindle‐shaped. In this case, however, the shape was roundish, oval or poorly defined. Ultrastructurally, the classic description was electron‐dense long rods and needles orientating along the long axis of the “spindle”. In this case, the only electron‐dense particles were dot‐like with a haphazard arrangement.

Supernatant K+ and in vitro haemolysis of washed red blood cells stored in saline for 3 days.

Washed Red Blood Cells (WR8C) is allowed a shelf-life of 24 hours. This study is undertaken to determine whether 1) the K+ level in the supernatant at 24 hours is acceptably-low for 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 of various storage age washed manually, and 4 units washea by Haemonetics were studied. Samples taken from each were divided into aliquots and tested for K+ level (mmol/1) and % loss of haemoglobin (Hb) in the supernatant at 6, 24, 48 and 72 hours. Results were as follows: red cells hours of storage age (n) 6 24 48 72 Manual wash 4-7 days K 0.7 4.8 9.2 13.1 (7) Hg 1.4 1.7 2.1 2.5 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 (8) Hg 2.6 4.0 4.6 5.0 Haemonetics 6-12 days K 0.2 0.8 3.9 / (4) Hg 1.3 2.6 3.8 / 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 for better preservation.

The type & Screen for pre-transfusion compatibility testing: Experience in a major teaching hospital in Hong Kong

Since December, 1986, the conventional crossmatch (CXM) has been replaced by the Type S Screen (T&S) for pre-transfusipn compatibility testing at the Prince of Wales Hospital. In order to appraise the T&S system and its practicability in Hong Kong, we compared it with the CXM according to the following parameters: proficiency in detecting antibodies, issuing of blood in emergency, level of technical difficulty, crossmatch-transfusion (C/T) ratio, reagent cost, workload, and blood outdating We found that the frequency and specificities of red cell antibodies detected by T&S were comparable to those in previous years when CXM was used. The T&S and CXM were also comparable in the issuing of blood during emergencies, and in the level of technical difficulty. The T&S reduced the C/T ratio from 3.25 in 1986 to 1.53 in 1988. The T&S entailed additional reagent cost, but saved on technologist’s workload. The main contribution of T&S was the dramatic reduction of outdated blood, from 1151 units in 1987 to 89 units in 1988.

Intranuclear inclusions in myeloma cells in a case of nonsecretory multiple myeloma

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 nonsecretory 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 compatible 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 of paraprotein in multiple myeloma which is rare, and the absence of membrane enclosing these intranuclear inclusions which is heretofore unreported, is probably not co-incidental but causally related. In this instance, the paraprotein which was produced in the nucleus and 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

Gelatinous transformation of marrow is a rare disease entity described in cachexia and various other disorders. Its association with systemic lupus erythematosus (SLE) has never been reported. We found gelatinous transformation in 3 of 30 patients with SLE with pancytopenia. Two of these patients were cachetic, one of whom also had active tuberculosis. We propose that gelatinous transformation can be associated with SLE, and its detection calls for an evaluation of nutritional status, and a search for chronic debilitating infections such as tuberculosis.

A SURVEY OF FRESH FROZEN PLASMA USE IN A TEACHING HOSPITAL IN HONG KONG

&NA; In response to an acute shortage of fresh frozen plasma (FFP), a survey of its use was conducted in our hospital. The survey was designed to separate the use of FFP into “appropriate use” and “inappropriate use” categories. Whether the use of FFP in a case could be assigned to “appropriate use” category or not was decided by pre‐set criteria based on the consensus statement1 published by the United States National Institute of Health (NIH). We found that during a 30‐day period, out of 746 units of FFP used, only 65 (8.7%) could be considered inappropriate use. Most of the FFP (67.6%) was used for liver disease with bleeding and/or abnormal coagulation tests, and for disseminated intravascular coagulopathy. In the “inappropriate use” category (8.7%), the leading causes were plasmapheresis, and hepatobiliary disease with normal coagulation tests and no abnormal bleeding.