PB1840 ACQUIRED HEMOPHILIA AND MONOCLONAL GAMMOPATHY OF HEMOSTASIC SIGNIFICANCE
Authors: Padilla I. , Martínez‐Robles V. , Melón A. , Castellanos M. , Rodríguez‐García J.A. , Escalante F. 
Published: June 2019

Background:

Acquired hemophilia A (HAA) is a rare hemorrhagic disease characterized by the appearance of autoantibodies against circulating factor VIII (FVIII). It has been described mainly in two groups: women of childbearing age and in older than 50 years, related to a very heterogeneous group of entities that include: drugs, autoimmune or neoplastic diseases. Within the hematological neoplasias, the one that has been most related is the chronic lymphocytic leukemia

Aims:

We present a very rare case of adquired A Hemophilia due wiirh an abnormal paraprotein.

Methods:

A 76‐year‐old man entered the Hematology department due to a month‐long evolution of hematoma and anemic syndrome. On physical examination, he had scattered bruises all over the body surface, especially in the extremities; with signs of deep bleeding in right upper and lower left limb, which implied limitation for mobility.

Complementary tests performed:

‐ Hemograme:. Hb 8.5 g / dL, normocytic and normochromic.

‐ Coagulation study: APTT ratio of 2.8 and an FVIII of 0.7%. The inhibitor titre was 6.2 UB / ml.

‐ Biochemistry was normal (including creatinine and calcium; and anemia study).

‐ A triple monoclonal band of 2.01 g / dL was observed in electrophoresis (0.56 + 1.08 + 0.37 g / dL). IgA of 1668 mg / dL (with decrease in IgG and IgM) and a serum free light chain (lambda / kappa) ratio of 21. The monoclonal component had remained stable up to six months before admission, when a third peak appeared [Table 1].

‐ In 24‐hour urine, he presented a proteinuria of 0.21 g (all Bence‐Jones lambda).

‐ A low‐dose whole‐body CT was also performed, which ruled out lytic lesions or underlying tumors.

Treatment with Cyclophosphamide and Prednisone was started. The response was good, with inhibitor reduction (5.6 UB), increased factor dosage (13%) and recovery from anemia. This allowed to perform bone marrow aspiration, which was compatible with multiple myeloma, with 13% of plasma cell. Cyclophosphamide was temporarily suspended due to an infectious disease. FVIII rise to 136%. After it, FVIII dropped to 18% and the MB rose to 1.2 g / dL (with a third peak of 0.6 g / dL). Cyclophosphamide was reintroduced and the dose of Prednisone increased again. Currently, the patient is asymptomatic, with no recurrence of anemia, with 90% FVIII and a monoclonal component of 1.5 g / dL; In addition, the FLC ratio has risen to 85. Continue close monitoring in our consultations.

Results:

The sudden onset of large bruises or extensive bruising in an adult patient with no history of bleeding disorder, should make us think of an acquired inhibitor of FVIII.The diagnosis should be based on the basic coagulation tests (lengthened APTT together with normal PT), the time correction in the Mixture Test, factor VIII dosage and Bethesda titulation.

The treatment of HAA should consist, first of all, in the control of bleeding. Once the hemorrhage is controlled, next objective must be the elimination of the inhibitor by means of immunosuppressants. In the limited available evidence, it seems that the best results are obtained with Prednisone in combination with Cyclophosphamide.

Summary/Conclusion:

The association of HAA and myeloma is extremely rare, with only five cases published.

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For the most part, the diagnosis of both pathologies was carried out concomitantly; and the treatment consisted, first of all, in trying to control the hemorrhagic clinic (by means of FVIII or FVIIr concentrates), and, subsequently, the start of the treatment directed to reduce paraprotein with antimyeloma treatment.