[CIS PIDD] [cis-pidd] lymphohistiocytosis vs ALPS?

[Wilmer Cordova Calderon]

Best regards
In Lima Perú find this case that 2 years ago no find a specific diagnostic
and child  disease is progressing and complicating other non-immune areas.
We will be eternally grateful to your help and collaborating diagnosis.
Thamk you.

Problems:
1.        Recurrent pancytopenia associated
2.        frequent infections (Pneumonia)
background:
physiological
Psychomotor development 4m, sat at 7-8m, yearling way.
Current treatment:
1. multivitamin Forkids
2. Captopril ¼ c / 24h-heart failure secondary portal hypertension.
Hospitalizations
1. 2nd 9m pneumonia and anemia Hb 4g%, thrombocytopenia 5d Huancayo
2. 3rd 6m pneumonia and anemia thrombocytopenia hepatosplenomegaly 7d
Huancayo
3. Pneumonia anemia thrombocytopenia 6m 4th cervical lymphadenopathy 5d
Huancayo
4. 5th pneumonia and anemia thrombocytopenia 5d Huancayo Transfusion PG:
5. 5th (October 2014) purulent lymphadenopathy, anemia, thrombocytopenia
CMV, HTP, dilated heart disease and anemia Transfusion 5d PG:
infections:
1. Intestinal infections 3v the first year. furoxone antibiotic.
2. SOB 1ª6m.
Metabolic studies. Liver biopsy with normal results.
Transfusions: PG 4 chances.
02/11/2015
October 2014 Table 1. Respiratory, painful cervical lymphadenopathy,
content purulent drainage, PCR for CMV 394 copies / ml (positive> 200),
pancytopenia (leukocytes 3150, HB 6.6, platelets 107, 000, creat 0.31, FA
1423, reticulocytes 4 %) fever, jaundice.
Eco doppler: HT Portal.
TEM TAP: hepatosplenomegaly (liver 164mm, 215mm spleen), liquid
perisplenic, multiple retroperitoneal lymph nodes, mesenteric, iliac and
inguinal up to 15mm.
Cervical Doppler ganglion formations with central and peripheral
vasculature, reagent aspect, in the context of lymphoproliferative aspect.
Pulmonary moderate to severe dilated cardiomyopathy, pericardial effusion,
mild systolic dysfunction FE 55% PSAP 62 mmHg HT echocardiography.
Treatment: Meropenem and vancomycin; Intravenous Ganciclovir, with
improvement of lymphadenopathy and infectious picture.
•        Biopsy of cervical and inguinal lymphadenopathy: Lymph node with
reactive changes predominance of hyperplasia and sinus histiocytosis T zone
with occasional eritrofagocitosis. Fibrosis; discrete architectural
distortion. CMV neg, CD20 + lymph B, CD3 + in Lymphoma T, DTT neg, CD10 +
in follicular centers, Ki67 + cells in replication.
Nonspecific reactive lymphoid hyperplasia, immunohistochemistry negative
for lymphoid neoplasia, immunoreaction for LMP1 negative.
Control abdominal Ecodoppler: higado135mm, spleen 166mm.
CMV viral load <200 copies / ml
Dilated cardiomyopathy, mild systolic dysfunction FEvi55% HT moderate
pulmonary PSAP 47 mmHg
02.11.15 Cold symptoms. Lymphadenopathy multiple cervical, axillary and
inguinal approx 2cm phones.
Diagnosis
1. Primary Immunodeficiency Vs High.
2. d / c Lymphohistiocytosis recurrent infectious reactive vs ALPS
3. Secondary hypersplenism
4. Hypertension Portal
5. Pulmonary Hypertension
6. dilated cardiomyopathy.
3.        CMV infection treated

Marzo 14
Linfos totales        1630
Cd4        349        21%
Cd8        929        57%
Cd3        1331
Linfos T cd45 cd3        1331        81
Cd19        57        3%
NK                45
nkt                3.4
Abril 14
IgA        168
IgG        3177
IgM        282
IgE        8

-- 
*Atte *
Wilmer Córdova
*Inmunología Alergología*
*Instituto Nacional de Salud del Niño - Perú*
http://www.isn.gob.pe
wcordova at insn.gob.pe
facebook.com/wilmer.cordova.71
twitter: @wilmer_cordova

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