HIV and Refractory Anemia With Excess Blasts (RAEB)

Geoffrey A. Modest,1,2 Timothy P. Cooley,3 and Jeff F. Zacks 4

1Boston Medical Center and Boston University School of Medicine, Boston, Massachusetts2Upham’s Corner Health Center, Dorchester, Massachusetts

3Sections of Hematology and Oncology, Boston Medical Center and Boston University School of Medicine, Boston, Massachusetts4Mallory Institute of Pathology, Boston Medical Center and Boston University School of Medicine, Boston, Massachusetts

We report the case of a 36 year old man who was hospitalized with pneumonia andpancytopenia with refractory anemia with excess blasts confirmed by bone marrow bi-opsy. He was subsequently found to have advanced HIV infection. Both the HIV infectionand the myelodysplastic syndrome responded to highly active anti-retroviral therapy(HAART) with sustained normalization of his hematologic abnormalities within 79 days.Am. J. Hematol. 70:318–319, 2002. © 2002 Wiley-Liss, Inc.

Key words: refractory anemia with excess blasts; myelodysplastic syndrome; HIV; AIDS

We report an unusual initial presentation of HIV dis-ease in a man with refractory anemia with excess blasts.A 36-year-old Haitian male, who had been treated inanother state 6 months previously for lobar pneumonia,was evaluated in our emergency room for fever, a non-productive cough, and dyspnea. Physical exam showed atemperature of 100.4 F, blood pressure of 139/88 mmHg,pulse 127, respiratory rate 24, and O2 saturation of 100%on oxygen by nasal cannula, with clear lung exam and

*Correspondence to: Geoffrey A. Modest, M.D., Clinical Professor ofMedicine, Boston Medical Center, and Boston University School ofMedicine, Boston, MA, and Medical Director, Upham’s Corner HealthCenter, Dorchester, MA. E-mail: gmodest@partners.org

Received for publication 12 December 2001; Accepted 15 March 2002

Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.10113

TABLE I.

Atpresentation

After 7 daysof treatment

After 14 daysof treatment

After28 days

of treatment

After42 days

of treatment

After79 days

of treatment

After6 months

of treatment

After9 months

of treatment

Weight (lbs) 198.5 210 211 220 218 239 246HIV viral load

(copies/mL)(lost) 3038 <400 <400 <50

CD4 count(per mm3)

2 90 115 240 350

WBC (×1,000) 1.9 3.5 4.2 4.4 5.9 6.6 6.9 8.1Smear 24% bands 1% metamyelocytes; 3% metamyelocytes; 24% polys 19% polys 15% polys 20% polys 16% polys

16% bands 5% bands 58% lymphs 70% lymphs 76% lymphs 70% lymphs 72% lymphsPlatelet count

(×1,000)12 35 38 66 129 232 250 280

Hematocrit (%) 32 32.8 34.6 35.6 36.7 40.8 43.9 43.6

Fig. 1. Low-power photo (original magnification 10×, he-matoxylin–eosin stain) showing hypercellular bone marrowwith erythroid hyperplasia and increased megakaryocytes.[Color figure can be viewed in the online issue, which isavailable at www.interscience.wiley.com.]

American Journal of Hematology 70:318–319 (2002)

© 2002 Wiley-Liss, Inc.

multiple lymph nodes up to 2 cm in diameter in thesubmandibular region. His initial laboratory results arenoted in Table I. Chest X ray confirmed a right lowerlobe infiltrate. Abdominal, chest, and pelvic CT scansshowed lymphadenopathy in the axillary, inguinal, retro-peritoneal, mediastinal, and pelvic areas without spleno-megaly. He was treated with ceftriaxone and azithromy-cin with resolution of his pneumonia. Because of hispancytopenia, bone marrow aspiration was performed,revealing myelodysplasia with 20% blasts (see Figs.1–4). Although the patient initially denied HIV risk fac-tors and stated that he had recently had a negative HIVtest, it was subsequently discovered that his wife wasHIV positive. With appropriate consent, the patient

tested positive for HIV antibody. He was started onhighly active antiretroviral therapy (HAART) with stavu-dine 40 mg BID, lamivudine 150 mg BID, and efavirenz600 mg at bedtime. He tolerated these medications well,with laboratory values noted in the table below. Of note,his peripheral blood responded dramatically within 7days of therapy, with normalization by day 79.

This unusual case raises a few issues. First, his pre-sentation for HIV infection was the unusual myelodys-plastic syndrome, refractory anemia with excess blasts(RAEB). A review of the National Library of Medicine’sMEDLINE and AIDSLINE databases revealed only onereference to RAEB in the setting of HIV [1], prior to theadvent of highly active anti-retroviral therapy. Second,there have been no reports to our knowledge of success-ful anti-retroviral therapy correcting this underlying, ag-gressive, and untreatable myelodysplastic syndrome witha typical median survival of only 5 months [2,3]. Asnoted in Table I, our patient’s bone marrow respondedpromptly and in all cell lines to anti-HIV therapy. Histreatment regimen did not include AZT, which weavoided because of his severe bone marrow suppression.

REFERENCES

1. Garavelli PL, Rossi G. Refractory anemia with excess of blasts in aHIV-positive patient [Italian]. Minerva Med 1990;81(7–8 Suppl):85–86.

2. Goasguen JE, Bennett JM. Classification and morphologic features ofthe myelodysplastic syndromes. Semin Oncol 1992;19:4.

3. Foucar K, Langdon RM II, Armitage JO, et al. Myelodysplastic syn-dromes: a clinical and pathologic analysis of 109 cases. Cancer 1985;56:553

Fig. 2. High-power photo (original magnification 40×)showing atypical dysplastic megakaryocyte.

Fig. 3. Medium-power photo (original magnification 20×)showing reticulin stain with mild increase in reticulin. [Colorfigure can be viewed in the online issue, which is availableat www.interscience.wiley.com.]

Fig. 4. Medium-power photo (original magnification 20×,with periodic acid Schiff (PAS) stain) showing myeloid leftshift, ALIP (abnormal location of precursors, compatiblewith myelodysplasia), and dysplastic megakaryocytes.[Color figure can be viewed in the online issue, which isavailable at www.interscience.wiley.com.]

Case Report: HIV and Refractory Anemia With Excess Blasts (RAEB) 319