Acquired Glanzmann’s thrombasthenia associated with Hairy cell leukaemia

Abstract

In the present report, a patient with Hairy cell leukaemia (HcL) who presented with bleeding diathesis was diagnosed to have acquired Glanzmann's thrombasthenia (GT). To our knowledge, this is the first report of acquired GT associated with HcL. In brief, HcL is a lymphoproliferative disorder involving the bone marrow and spleen [1] and GT is a defect caused by abnormality in GPIIb ⁄ IIIa receptor [2]. Acquired GT is an uncommon event in association with chronic limphoproliferative disorders. This may be caused by the formation of autoantibodies against GPIIb ⁄ IIIa complex [3]. This study is on 51-year-old male patient who presented with bleeding diathesis from gum and nose; he occasionally had Gastro-Intestinal bleed. Presence of hairy cells was confirmed using peripheral smear blood and bone marrow analysis and subsequently using electron microscopy. Morphological analysis revealed presence of hairy cells highlighting the irregular cytoplasmic projections of the abnormal mononuclear cells. Non-aggregative, isolated platelets were observed in peripheral smear of blood. Basic coagulation study showed presence of adequate platelet count (210 · 10 3 per lL), normal prothrombin time, activated partical thromboplastin time and prolonged bleeding time (15¢) in-patient. Unlike patients with acquired Bernard Soulier Syndrome and other acquired platelet function disorders, this patient had normal platelet count throughout the observation. Moreover, he did not have spenomegaly or neutropenia. Platelet aggregation studies revealed absent aggregation with all four agonists ADP (0%), ADR (0%), Arachidonic acid (0%) and collagen (0%), which depend on GPIIb ⁄ IIIa receptor and normal aggregation with Ristocetin (70%) that depends on GPIb receptor. These features are similar to those usually seen in GT (a) (b) Figure 1 (a) Analysis of GPIIb ⁄ IIIa by Flow cytometry using FITC-labelled CD41 and CD61 and (b) Analysis of individual, total GPIIb and GPIIIa protein by Western blot followed by chemiluminescent method in patient as well as in the normal controls.

An Unusual Cause of Bleeding in a Patient with Chronic Myeloid Leukemia Chronic Phase

“…An extensive review of the literature revealed that very few cases of acquired GT have been reported in association with neoplasms of lymphoid origin such as multiple myeloma, non-Hodgkin's lymphoma, and Hodgkin's disease [13]. Kannan et al found a case of hairy cell leukemia associated with acquired GT [14]. Although bleeding diathesis attributed to defective platelet function is not uncommon in CML, typical GT (e.g., a defect in platelet aggregation) has not been reported earlier in adult CML.…”

A unique phenotype of acquired Glanzmann thrombasthenia due to non‐function‐blocking anti‐αIIbβ3 autoantibodies

“…Porcelijn [3] 67, F ITP post-splenectomy (+)IgG1, Ib/IX Normal Porcelijn [3] 51, F ITP post-splenectomy (+)IgG1 Normal Kannan [43] 51, M Hairy cell leukemia Not directly detected Decreased by FCM, normal by WB Solh [16] 52, F Hypothyroidism (+), Ib/IX, IaIIa Decreased by some antibodies Raman [44] 44, M HL (+) n.d. Blickstein [45] 75, F SLE, antiphospholipid antibody syndrome, ITP postsplenectomy Not directly detected n.d.…”