Autoimmune thrombocytopenic purpura with spuriously normal platelet count and ?punch-hole? red cells

Abstract Accumulation of a lipid, histochemically a phospholipid, in the histiocytes of the splenic pulp was observed in seven patients with thrombocytopenic purpura. Six had classical idiopathic thrombocytopenic purpura with abundant megakaryocytes in the bone marrow. Splenectomy resulted in clinical and hematologic remissions in four of these six, continued thrombocytopenia in the fifth, and in the continued requirement of corticosteroid to maintain a reasonably normal platelet count in the sixth. The seventh patient, who died shortly after splenectomy, had marked hypoplasia of megakaryocytes. Similar lipid accumulation was not seen in more than 700 other spleens, removed for a variety of reasons, reviewed in this study. Platelet phagocytosis has been suggested as a source of the lipid.

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ADAMTS-13 Levels Do Not Predict Outcome in Thrombotic Thrombocytopenic Purpura: Protein S Levels Are Maintained during Plasma Exchange with Solvent Detergent Treated Plasma.

Blood ◽

10.1182/blood.v110.11.2895.2895 ◽

2007 ◽

Vol 110 (11) ◽

pp. 2895-2895

Author(s):

Gail A. Rock ◽

David Anderson ◽

Barrett Benny ◽

David Barth ◽

William Clark ◽

...

Keyword(s):

Molecular Weight ◽

Enzyme Activity ◽

Platelet Count ◽

Plasma Exchange ◽

Protein S ◽

Inhibitor Activity ◽

Thrombocytopenic Purpura ◽

Normal Platelet Count ◽

Normal Platelet ◽

Alternative Solutions

Abstract Introduction The ultimate therapy for TTP is not yet defined. While approximately 85% of patients respond to plasma exchange with FFP, the use of alternative solutions such as cryosupernatant plasma, which is deficient in the high molecular weight forms of vWF. has not been proven by appropriate randomized prospective sample trials. However, it is intellectually appealing. Additionally, the use of the ADAMTS 13 level either to predict disease or outcome has not been resolved. Methods Cryosupernatant plasma (CSP) was compared to solvent detergent treated plasma (SDP) over one cycle (nine days) of plasma exchange (PE) and subsequent procedures as required. Throughout treatment, ADAMTS 13 and protein S levels were followed. Results At the primary end point of one month, 3 of 35 of the CSP and 1 of 27 of the SDP patients died. The average platelet count was 184 × 109 per litre for CSP and 209 × 109 per litre for SDP. While vWF and FVIII were elevated in all patients at entry, no unusually large vWF multimers were seen at any time. At entry 9 patients had ≤ 0–10% ADAMTS 13 and 14 of 62 had normal levels with the rest in between. At presentation only 9 patients had 100% inhibitor activity with no inhibitor in 18 patients. Of the four patients who died, two who received CSP, had 100% enzyme activity at presentation whereas one had no ADAMTS-13 and another had 10%. All 4 patients died within 10 days. Fifteen patients with less than 10% enzyme activity had >1.5 vWF. However, elevated (>3u/mL) vWF was seen in the presence of normal ADAMTS-13 levels. Six months after remission, and in the presence of a normal platelet count, ADAMTS13 remained low in 5 patients and normal in 15.Protein S levels in 41/62 patients studied showed little variation during therapy with values on day 3 and 5, similar to those seen on entry. Conclusion In idiopathic TTP, ADAMTS 13 levels do not correlate with disease, and are not predictive of outcome. While protein S levels in the SDP were lower than those found in FFP, this did not appear to have clinical significance.

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Splenectomy during cesarean section in a patient with severe idiopathic thrombocytopenic purpura (ITP) refractory to the medical treatment

Acta chirurgica iugoslavica ◽

10.2298/aci1404065g ◽

2014 ◽

Vol 61 (4) ◽

pp. 64-66

Author(s):

Andreja Glisic ◽

Aleksandar Cetkovic ◽

Slavko Matic ◽

Milos Petronijevic ◽

Ivo Elezovic

Keyword(s):

Platelet Count ◽

Cesarean Section ◽

Idiopathic Thrombocytopenic Purpura ◽

Reproductive Age ◽

Women Of Reproductive Age ◽

Thrombocytopenic Purpura ◽

Normal Platelet Count ◽

Normal Platelet ◽

Healthy Female ◽

Discharge From Hospital

Idiopathic thrombocytopenic purpura (ITP) is a disease of women of reproductive age and accounts for 5% of pregnancy associated thrombocytopenia. We reported the case of 21-year-old primigravida with ITP who was admitted to our hospital at the 28th weeks of gestation with platelet count of 22x109/l. Despite medicament treatment (prednisone, intravenous immunoglobulin /IgG/) there were falls in the platelet count, last at the 33rd week of gestation when platelet count was 15x109/l. In consultation with hematologist and surgeon we decided to deliver the patient by cesarean sec- tion during which splenectomy will be done. Patient received 60g of intravenous IgG two days before operations which were performed at the 34th week of gestation. Healthy female infant was born with normal platelet count and maternal platelet count at the discharge from hospital was 346x109/l.

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Fatal cerebral hemorrhage in a patient with thrombotic thrombocytopenic purpura with a normal platelet count during treatment with caplacizumab

Platelets ◽

10.1080/09537104.2021.1922883 ◽

2021 ◽

pp. 1-2

Author(s):

Kim Ditzel ◽

Dirk Jan Mons ◽

Rob Fijnheer

Keyword(s):

Platelet Count ◽

Thrombotic Thrombocytopenic Purpura ◽

Cerebral Hemorrhage ◽

Thrombocytopenic Purpura ◽

Normal Platelet Count ◽

Normal Platelet

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Thrombotic Thrombocytopenic Purpura: Mechanism for Effectiveness of Plasmapheresis

10.1055/s-0038-1665810 ◽

1979 ◽

Author(s):

J. G. Kelton ◽

P. B. Neame ◽

I. Walker ◽

A. G. Turpie ◽

J. McBride ◽

...

Keyword(s):

Platelet Count ◽

Thrombotic Thrombocytopenic Purpura ◽

The Other ◽

Unknown Etiology ◽

Normal Range ◽

Antiplatelet Antibody ◽

Thrombocytopenic Purpura ◽

Serious Illness ◽

Normal Platelet ◽

Very High

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious illness of unknown etiology. Treatment by plasmapheresis has been reported to be effective but the mechanism for benefit is unknown. We have investigated the effect of plasmapheresis in 2 patients with TTP by quantitating platelet associated IgG (PAIgG) levels prior to and following plasmapheresis. Both patients had very high levels of PAIgG at presentation (90 and A8 fg IgG/platelet respectively, normal 0-5). in both, the PAIgG levels progressively fell to within the normal range and the platelet count rose following plasmapheresis. One patient remained in remission with normal platelet counts and PAIgG levels. The other relapsed after plasmapheresis and the PAIgG level rose prior to the fall in platelet count. Plasmapheresis was repeated and resulted in normalization of both the platelet count and PAIgG level. It is suggested that plasmapheresis removes antiplatelet antibody or immune complexes which may be of etiological importance in this illness.

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Cancer incidence in patients with a high normal platelet count: a cohort study using primary care data

Family Practice ◽

10.1093/fampra/cmy018 ◽

2018 ◽

Vol 35 (6) ◽

pp. 671-675 ◽

Cited By ~ 9

Author(s):

Emily Ankus ◽

Sarah J Price ◽

Obioha C Ukoumunne ◽

William Hamilton ◽

Sarah E R Bailey

Keyword(s):

Primary Care ◽

Cohort Study ◽

Platelet Count ◽

Cancer Incidence ◽

Normal Platelet Count ◽

Normal Platelet ◽

Primary Care Data

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Thrombotic Thrombocytopenic Purpura: Mechanism for Effectiveness of Plasmapheresis

10.1055/s-0039-1684538 ◽

1979 ◽

Author(s):

J.G. Kelton ◽

P.B. Neame ◽

I. Walker ◽

A.G. Turpie ◽

J. McBride ◽

...

Keyword(s):

Platelet Count ◽

Thrombotic Thrombocytopenic Purpura ◽

The Other ◽

Unknown Etiology ◽

Normal Range ◽

Antiplatelet Antibody ◽

Thrombocytopenic Purpura ◽

Serious Illness ◽

Normal Platelet ◽

Very High

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious illness of unknown etiology. Treatment by plasmapheresis has been reported to be effective but the mechanism for benefit is unknown. We have investigated the effect of plasmapheresis in 2 patients with TTP by guantitating platelet associated IgG (PAIgG) levels prior to and following plasmapheresis. Both patients had very high levels of PAIgG at presentation (90 and 48 fg IgG/platelet respectively, normal 0-5). In both, the PAIgG levels progressively fellto within the normal range and the platelet count rose following plasmapheresis. One patient remained in remission with normal platelet counts and PAIgG levels. The other relapsed after plasmapheresis and the PAIgG levelrose prior to the fall in platelet count. Plasmapheresis was repeated and resulted in normalization of both the platelet count and PAIgG level. It is suggested that plasmapheres is removes antiplatelet antibody or immune complexes which may be of etiologicalimportance in this illness.

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Regulation of megakaryocyte ploidy in vivo in the rat

Blood ◽

10.1182/blood.v75.1.74.74 ◽

1990 ◽

Vol 75 (1) ◽

pp. 74-81 ◽

Cited By ~ 32

Author(s):

DJ Kuter ◽

RD Rosenberg

Keyword(s):

Platelet Count ◽

Feedback Loop ◽

Antiplatelet Antibody ◽

Normal Platelet Count ◽

Normal Platelet ◽

Plasma Factor ◽

Ploidy Changes ◽

The Relationship ◽

Made In

Abstract The relationship between the bone marrow (BM) megakaryocyte and the circulating platelet was explored. Incremental changes in platelet count were made in rats by infusion of antiplatelet antibody or by platelet transfusion, and the response of megakaryocytes was measured by flow cytometry. Proportional changes in megakaryocyte ploidy were demonstrated: As the platelet count declined, ploidy increased; as the platelet count increased, ploidy decreased. Even moderate degrees of thrombocytopenia and thrombocytosis (48% and 177% of the normal platelet count) were associated with changes in ploidy. These changes were not the results of the technique used to alter the platelet count because reinfusion of platelets after 3 hours of thrombocytopenia prevented any ploidy change. These studies proved that the circulating platelet and the megakaryocyte constitute a classic feedback loop whose activity can be measured by the degree of ploidization of the megakaryocyte. The minimal duration of thrombocytopenia necessary to promote megakaryocyte ploidy changes was approximately 10 hours. Using a BM culture assay, we identified a plasma factor which induced alterations in megakaryocyte ploidy and whose level is inversely proportional to the platelet count.

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Quantitation of platelet-associated IgG by radial immunodiffusion

Blood ◽

10.1182/blood.v57.4.809.809 ◽

1981 ◽

Vol 57 (4) ◽

pp. 809-811 ◽

Cited By ~ 24

Author(s):

BS Morse ◽

D Giuliani ◽

M Nussbaum

Keyword(s):

Platelet Count ◽

Immune Thrombocytopenic Purpura ◽

Radial Immunodiffusion ◽

Thrombocytopenic Purpura ◽

Platelet Counts ◽

Normal Platelet ◽

Serum Igg ◽

Acute Itp ◽

Igg Level

Abstract Platelet-associated IgG (PAIgG) was measured by a simple radial immunodiffusion technique using washed solubilized platelets and commercially available immunoplates. Subjects with normal platelet counts had PAIgG levels of 1.5--7.0 fg/platelet. Subjects with idiopathic immune thrombocytopenic purpura (ITP) had levels ranging from 5.7 to 70.5 fg/platelet. All patients with recurrent ITP and 85% of patients with acute ITP had elevated PAIgg. Elevated PAIgG was also found in 17% of patients with recovered ITP, 40% of patients with SLE and thrombocytopenia, 57% of patients with thrombocytopenia occurring during the course of septicemia, and 100% of patients with IgG myeloma in whom the serum IgG level was clearly elevated, regardless of the platelet count. The results are similar to reports that used more complex techniques.

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